AIN SHAMS UNIVERSITY

Bone Tumors

Mohamed Sobhy

Quick Guide

Bone Tumor Diagnosis

Different Bone Tumors

Tumor Like Conditions

Soft Tissue Tumors

Bone Tumors

Index

Table of Contents
Tumor Principle...................................... 1-12
Myelogenic Tumors ............................... 13-28
Fibrous Tumors ..................................... 29-40
Osteogenic Tumors ............................... 41-62
Chondrogenic Tumors .......................... 63-75
Cystic Lesions ........................................ 77-93
Miscellaneous ........................................ 95-100
Soft Tissue Sarcomas ............................ 101-123

Tumour Principles
Staging Musculo-skeletal Neoplasms (Enneking)
Classification based on:
1. Histological grade (G)
2. Site (T)
3. Metastases (M)
Enneking staging system was tested retrospectively on 397 cases of bone & soft tissue tumours.
Shown that prognosis varied with the stage.
The system aids in assessing prognosis & planning management
NB: Applies to lesions of connective tissue, not primary lesions of round cell origin (eg
leukaemia's, lymphomas, myeloma or Ewings)
Enneking's Surgical Stages
STAGE GRADE
SITE

METASTASES

1A
1B
2A
2B
3

None(M0)
None(M0)
None(M0)
None(M0)
Yes(M1)

Low(G1)
Low(G1)
High(G2)
High(G2)
Low(G1) or
High(G2)

Intracompartmental(T1)
Extracompartmental(T2)
Intracompartmental(T1)
Extracompartmental(T2)
Intracompartmental(T1) or
Extracompartmental(T2)

Grade (assessment of biological aggressiveness)

G0 Histologically benign (well differentiated and z cell / matrix ratio)
G1 LG malignant (zmitoses, moderate differentiation, local spread & z metasases
G2 HG malignancy (frequent mitoses, poorly differentiated); High risk of metasases
Features of aggressive tumours:
Cellular atypia
Frequent mitoses
Extensive necrosis
Significant vascularity
Small amounts of immature matrix
Examples:
LOW (G1)

Parosteal osteosarcoma
LG medullary osteosarcoma
Giant Cell Tumour
Secondary Chondrosarcoma
Fibrosarcoma
Myxoid Liposarcoma

Clear cell tumour of tendon sheath

Chordoma
Adamantinoma

HIGH (G2)

classic osteosarcoma
radiation osteosarcoma
Paget's osteosarcoma
Primary Chondrosarcoma
Malignant Fibrous Histiocytoma
Pleomorphic Liposarcoma
Neurofibrosarcoma (Shwannoma)
Rhabdomyosarcoma
Synovioma

Site (anatomic setting of the lesion)

T0 Intracapsular
T1 Intracompartmental (eg cortical bone, joint capsule or fascia)
T2 Extracompartmental (spreads beyond 'fascial' plane out longitudinal containment)
Intracompartmental

Extracompartmental

intraosseous
intra-articular
Intrafascial compartments:
ray of hand or foot
posterior or anterior leg
ant, med, post thigh
buttocks
volar or dorsal forearm
anterior or posterior arm
pericapsular

soft tissue extension

deep fascial extension
Extrafascial planes/spaces: (neurovascular
containing spaces)
mid & hind foot / mid hand
popliteal fossa
groin-femoral triangle
intra-pelvic
antecubital fossa
axilla
paraspinal

Metastasis (nodal or blood borne tumour spread)

M0 No evidence of regional or distant metastases
M1 Regional or distant metastases evident

Tumour Workup
1. Clinical examination (age, sex, site and past history)
o Thyroid
o Liver
o Breasts
o Kidney
o Chest
o Rectal (prostate & rectal tumours)
2. Bloods
o FBC (leukaemic cells etc)
o ESR (often elevated)
o Biochemistry (Ca++, PO4, liver enzymes and Alkaline Phosphatase) -> mets
o Acid Phosphatase (prostate and increased with metastatic deposits)
o Thyroid function tests
o PSA
o Serum Protein Electrophoresis (Myeloma)
3. Urinalysis
4. Urine Bence-Jones (myeloma)
5. CXR
6. Abdominal ultrasound
7. Bone scan other sites
8. MRI soft tissue extent and association with nerves and vessels
9. CT of lesion and chest ( staging)
10. Angiography tumour blood supply and relationship to major vessels
11. Biopsy

Diagnosis
1. AGE PREDILECTION
0 - 5yr
6-18yr

19-40y
40+yrs

Benign

Malignant

Other

1. Eosinophilic Granuloma (EG)

2. (Unicameral bone cyst- rare)
1. Unicameral Bone Cyst
2. ABC
3. Enchondroma
4. Osteo & Chondroblastoma
5. Nonoss&chondromyxoid fibroma
6. Chondromyxoidfibroma 7.EG
1. Giant Cell Tumour
2.EG

1.
2.
1.
2.

1. Osteomyelitis
2. healing/ stress fracture
1. Osteomyelitis
2. Fibrous Dysplasia
3. Osteofibrous Dysplasia

laeukaemia
Metast.Neuroblastoma
Ewings Sarcoma
Osteosarcoma

1. Ewings Sarcoma
1. Metastases
2. MM & Lymphoma
3. osteo,chondro,fibrosarcoma

1. Hyperparathyroidism
2. Osteomyelitis
3. Paget's

2. NUMBER OF LESIONS
Is it monostotic or polyostotic?
3. TUMOR LOCATION
Metaphyseal Diaphyseal Epiphyseal
Medullary (central, eccentric) - Cortical Juxta-cortical as:
parosteal osteosarcoma - no space bet lesion & cortex
myositis ossificans - space between lesion & cortex
Exostoses (cartilage capped) - shares cortex bone
4. LYTIC, SCLEROTIC, MIXED

5. TUMOUR GROWTH (LODWICK CLASSIFICATION)

GI geographic: a- well defined border & sclerosis
b- well defined border
c-ill defined border & cortex penetration
GII moth-eaten (intermediate growth)
GIII permeative (rapid Growth) very long or ill defined zone of transition
6. TUMOR MATRIX
Cartilage producing lesions = stippled calcification on x-ray Popcorn
Osteoid mineralization = Cloud Like Matrix (bone infarct - 'smoke up a chimney')
Fibrous dysplasia Ground Glass appearance of the fine spicules of dysplastic bone
7. EFFECT ON BONE: (Erosion, Expansion, Penetration, Trabeculation)
NB - describe lesion as aggressive/non-aggressive NOT malignant/benign
Non aggressive
lobulated erosion Scalloped Margin e.g. enchondromas
Trabeculation: 1- Displacement of residual trabeculae
2- Reactive new bone formation evoked by the near by tumor
Aggressive growth progressive endosteal erosion periosteal reaction Cortical Expansion
Cortex penetration reach & elevate periosteum codman/sunray/hair on end/ onion peel
8. RESPONSE OF BONE
HG spread too rapidly for the bone to respond wide zone of transition
LG bone has the time to react:
1. Narrow, sclerotic zone of transition
2. thickened periosteal response

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9. PERIOSTEAL REACTION:
1. Continuous:
1. Solid: osteoid osteoma
2. Linear: ABC, GCT
3. Lamellated: Onion peel
2. Interupted: Codmans U
3. Complex: Sun ray and Hair on end appearance
10. SOFT TISSUE MASS: usually denotes primary lesions
Site Predilection

Behavior Grading

Typical example

Classification
Benign/latent (self limiting)
Benign/active (grow capsule)
Benign/aggressive (no capsule)
Malignant/low-grade
Malignant/high-grade

Bone
Nonossifying fibroma
Aneurysmal bone cyst
Giant-cell tumor
Parosteal osteosarcoma
Classic osteosarcoma

Trabeculations

Lesion
Gian Cell Tumor
ABC
Chondromyxoid fibroma
Non ossifying fibroma
Hemangioma

Trabecular Pattern
Delicate and thing
Delicate & horizontal
Coarse and thick
Lobulated
Striated and radiating

Soft Tissue
Lipoma
Angiolipoma
Aggressive fibromatosis
Myxoid liposarcoma
MFH

RADIOLOGICAL FEATURES
Phemister's Law = commonest site of infection & tumours is ! fastest growing site of ! long bone
To see a lucent lesion in bone, an estimated 30 - 50 % of the bone must first be lost
Features of Malignancy:
Metaphyseal:
Expand bone:
Spinal Lesion:
1. size of lesion (small is
- adults metastases /
Simple (central)
Giant cell tumour
good, big is bad)
myeloma / hemangiomas
FCD (eccentric)
ABC
2. cortical destruction
- young:
Simple bone cyst
3. No sclerosis margin
body: histiocytosis
4. Soft tissue mass
arch: ABC /O.blastoma
osteoid osteoma.
-sacrum chordoma
osteoid osteoma
Enchondroma- speckled
Fibrous Dysplasia = a long
Giant cell tumours nearly
looks as Brodie's abscess
cyst, small bones
lesion in a long bone
always occur near a joint
Enchondroma, simple bone
surface (in mature bone)
cyst & fibrous dysplasia
can look very similar
Cartilage tumours - have speckled calcification
Lytic infection- mimics any
Histiocytosis X
Chondroblastoma= Giant cell tumour of children. Only tr
bone lesion.
(Eosinophilic granuloma)arising in the epiphysis
flat bones usually,
Chondromyxoid fibroma- older Pt., eccentric, speckled
commonest is skull
Hydatid- rural
Hyperparathyroidism- brown tumour, phalangeal erosions, sclerosis, subperiosteal bone resorption, osteitis fibrosa cystica
Certain bones in the body can be considered "epiphyseal equivalents" for purposes of differential diagnosis. These include
the patella, the calcaneus, and most apophyses. Therefore, for lucent lesions in these areas, one should include the classic
epiphyseal entities such as chondroblastoma (children), giant cell tumors (adults) and aneurysmal bone cysts.

Buzzwords:
Onion Peel
Punched-out lesion
Moth-eaten
Permeative pattern
Rat-bite lesions
Pencil in cup
Blow-out
Soap bubble
Nidus
Sunburst spiculation
Popcorn balls
Sled runner tracks
Bear bite
Smoke goes up a chimney
Gracile bones
Erlenmeyer flask
Scallops from without
Scallops from within
Ground glass
Salt & pepper skull
Stress lines
Looser's lines

Ewing Sarcoma
MM, Eosinophilic granuloma
Round cell tumours
Round cell tumours
Congenital syphilis
Aneurysmal bone cyst
Aneurysmal bone cyst
Giant cell tumour
Osteoid osteoma
Osteosarcoma
Cartilage tumours
Ollier's & Mafucci's
Fibrosarcoma
Medullary infarct
Osteogenesis imperfecta
Gaucher's disease
Neurofibromatosis
FD, NOF, SBC, enchondromas, chondromyxoid & chondrosarcoma
Fibrous dysplasia, enchondromas
Paget's disease
Paget's disease (on convex/ tension side of bone)
Rickets / osteomalacia (on concave/ compression side of bone)

MRI features:
most tumours are dark on T1 & bright on T2
Desmoids, scar tissue, fibrous tissue & cortical bone are dark on T1 & T2
Lipomas, liposarcomas & haemangiomas are bright on T1 & T2

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Tumour
Ewings Sarcoma
MM
RCS
Hodgkin
Lymphosarcoma
EG (BFH)
MFH
Fibrosarcoma
Desmoid
Osteosarcoma
Osteoid Osteoma
Osteoblastoma
Osteochondroma
MHE
Enchondroma
Chondroblastoma
Chondromyxoid
Chondrosarcoma
Giant cell Tumor
ABC
Adamantinoma
Chordoma

AGE
nd

2 decade
5th & 6th decade
5th & 6th decade
20-30
40-60

< 30y
10-30
10-30
<20y
3-12y
10-50
10-20
10-30
30-60

10-40
40-70

Origin
Endothelial elements of BM PNET
Plasma cells
BM lymphoid cells
Lymphoid reticulum
Lymphocytes
Histiocytes
Fibroblasts
Fibroblasts
Reaction to add.magnus stress
Bone forming mesenchymal cells
Unknown Reactive bone lesion
Unknown
Physeal herniation
AD & EXT 1&2 genes
chondroblasts
chondroblasts youngs GCT image
Physeal remnants
Cartilage forming cells
Supporting cells of bone CT
Aneurysmal malformation
Epithelial origin
Remnants of notochord

Rank
3rd common

2:1 (black) COMMONEST

1ry malign.BM

2:1
2:1
3:1

Rare
2nd Common

Commonest Benign

2:1
2:1

Oliers & Maffucci

2:1

Extremely rare
Rare

Eccentric metaphyseal
4th common sarcoma

chrornosomal abnormalities

RB I gene seen in both retinoblastoma and osteosarcoma

P53 tumour suppression gene is mutated in 50% of human tumours and nearly all types of
sarcomas including osteosarcoma, rhabdomyosarcoma and neurofibrosarcoma
NFl gene associated with neurofibrosarcomas neurofibromatosis, schwannoma
Chromosomal translocation t(11;22):(q24:q12) seen in Ewings sarcoma and PNETs
A variety of benign tumours has also been shown to have specific chromosomal alterations.

Lucent bone lesions = FOGMACHINES

Fibrous Dysplasia
Osteoblastoma
Giant Cell Tumour
Metastasis/ Myeloma
Aneurysmal Bone Cyst
Chondroblastoma/ Chondromyxoid Fibroma
Hyperparathyroidism (brown tumour)/ Haemangioma
Infection
Non-ossifying Fibroma
Eosinophilic Granuloma/ Enchondroma
Simple Bone Cyst

Sclerotic bone lesions = VINDICATE

Vascular
o hemangiomas
o infarct
Infection
o chronic osteomyelitis
Neoplasm
o primary
osteoma
osteosarcoma
o metastatic: (prostate, breast, Oat cell lung carcinoma)
Drugs
o Vitamin D
o fluoride
Congenital
o bone islands
o osteopoikilosis
o osteopetrosis
o pyknodysostosis
Endocrine/Metabolic
o hyperparathyroidism
o Paget's disease

Radiological Investigations
Bone Scan
Tc99m-HDP (Diphosphonates labelled Technetium 99) usually used in dose of 500 - 600 mBq
Osteoid osteoma -> increased activity in blood pool phase as well as delayed bony phase
Inflammatory lesions also increased activity in blood pool scan
Gallium 67 has also been utilised
CT Scans
Indicates extent of bony involvement / destruction
Good for detecting subtle cortical disruption, fracture, calcification or ossification
Not as good as MRI for soft tissue extent. Can detect soft tissue masses >/= 5mm diam.
Useful to stage - eg. lung secondaries
Angiography
Feeding vessels identified as well as the tumour proximity to major vessels
Displacement of vessels by tumour -> access for excision of the tumour
Embolisation of vascular tumour prior to surgery
Intra-arterial chemotherapy
MRI
Good soft tissue definition (better than CT) and extra-osseous extension, joint involvement, skip
lesions and epiphysial extension -> staging -> extent of tumour both intra and extra medullary
Able to image in any plane - thus good for pelvic/sacral lesions
T1 images best for looking at extent of BM involvement & T2 useful for evaluating cortical
bone and soft tissue extent (NB - CT better for showing areas of calcification / ossification)
Best technique to identify haemorrhage/oedema/inflammation- eg prior biopsy
Oedema usually surrounds malignant lesions & is unusual around benign tumours

Biopsy of Bone Tumours

Principles:
1. Should know probable diagnosis & stage before biopsy (it is the last step in pt staging)
2. Performed by the surgeon who will perform the definitive surgery (? biopsy -> frozen
section -> definitive surgery during the same anaesthetic)
3. Biopsy tract orientation & location is critical included in the definitive surgery if malignant
4. Meticulous haemostasis to avoid tracking haematomas
5. Send samples for microbiological analysis
Open Biopsy
Aim for excisional biopsy when possible esp. in benign lesions
Incisional biopsy preferable in malignant lesions
After consultation with the pathologist and radiologist
1. Longitudinal incision
2. Sharp dissection should proceed directly to the tr (via muscle not between muscle planes)
3. Uninvolved anatomic compartments should not be exposed
4. Avoid all major neurovascular structures to prevent contamination
5. Excise block of reactive tissue, pseudo capsule, capsule & tr block formalin +/- frozen
6. Windows in bone should be as small as possible and oval to avoid stress risers and
pathological fracture
7. Release tourniquet prior to closure haemostasis
8. Close with a subcut. stitch
9. Drains should come out through the wound
10. If proceed following biopsy new instruments and drapes to stop seeding
Needle Biopsy
1. As for open biopsy
2. Place the biopsy tract where it can be excised
3. Fine needle biopsy:
o relies on cytological interpretation by an experienced pathologist
o accuracy = 65 to 95% (determined by the adequacy of the collected tissue sample)
o does not allow for immunohistochemical analysis
4. Core needle biopsy:
o uses trocar cannula system, outer sleeve closes over the trocar to grap ! tissue
o provides more tissue than FNAC and allows for immuno-histochemical analysis
o accuracy = 75 to 95%
o A/E of needle biopsy = tissue may be from necrotic part not suitable for dx, or
tissue may be reactive not representative of tumor Frozen may be beneficial.
Frozen Section
1. Able to determine if specimen is adequate or representative
2. Can decide if lesion is inflammatory and needs culturing / need further investigations
3. Immediate diagnosis possible- can proceed to definitive surgery

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SURGICAL
PROCEDURES
(Aim:
remove the lesion minimal risk of local
recurrence)
Limb Salvage
Criteria for limb salvage:
1. local control of the lesion must
be at least equal to amputation
2. the saved limb must be
functional
Types of osseous resection
1. Inter calary (between joints)
2. Intra articular (one side of joint)
3. Extra articular (both sides of the
joint)
Various methods of salvage eg,
endoprosthesis, allograft, composite,
arthrodesis
Relative contraindications: Pathological
# & Skeletal immaturity
metastasis
Distal
is
not
a
contraindication
Choose anatomical location favours
reconstruction & allows wide margins
Surgical Margins:
1. Intra-lesional
o through the tumour & leaves
macroscopic tumour
o not therapeutic
2. Marginal
o Via
pseudo-capsule/reactive
zone (contains inflame.cells,
oedema, satellites of tr)
o controls non-invasive benign tr
o recurrence of malignant tumours
= 25-50%
3. Wide
o around reactive zone, leaving a
cuff of normal tissue
o skip lesions left
o recurrence of malig tr < 10%
4. Radical
o removal of entire compartment
or compartments
o distant metastases left
5. Amputation
o should be thought of as a form of reconstruction when function outweighs preservation

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ADJUVANT CHEMOTHERAPY
Nomenclature:
"Adjuvant" = chemotherapy given to ttt micrometastases
"Neoadjuvant" = preoperative chemotherapy before resection of 1ry tumor.

Neo-adjuvant chemotherapy
= Staging Chemotherapy Restaging Surgery Trr kill rate Further treatment
(change chemo or introduce radiotherapy as indicated)
Enables action of agents against tumour to commence immediately
Efficacy of chemotherapeutic agents evident at the time of tumour resection
Reduces the mass and vascularity of the tumour prior to definitive surgery
Enables time for operative planning
Want 90% kill rate and if < 90% change agents
No y survival evident with different agents in those who do not respond to the initial
treatment. (? just identifies those patients with a good prognosis).
Commence adjuvant treatment once the wound has healed
May persist for 2 months to 2 years depending on the response
Localised disease = 60-70% long-term disease-free survival
May lead to:
1. Stunting of growth (catch up later)
2. Osteoporosis
3. AVN
4. Cisplatinum nephro & ototoxicity
5. Adriamycin cardiotoxicity
6. Vincristine Neurotoxicity
7. Chemotherapeutic induced malignancy usually blood forming eg leukaemias but also
may Ca bladder or skin (particularly cyclophosphamide)
Methotrexate
Binds to dihydrofolate reductase and therefore blocks purine synthesis.
Potentiates the effects of radiotherapy
Citravorum factor "rescue" replenishes the folate pool
Adriamycin
Cytotoxic antibiotic
Also potentiates radiotherapy

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RADIOTHERAPY
Pre-operative radiotherapy may z size of the tumour, decreased chance of seeding at the time
of resection or if seeding z chance of viability of the shed cells
Effect of radiation
Direct = absorption by complex molecules causes rupture of chemical bonds with
damaging effects
Indirect = by ionisation & formation of highly reactive intracellular free radicals DNA
changes stop cell reproduction &z cell turnover loss of specific cell function
The effect on cancer cells not more rapid growth of cells but in the capacity for
recovery and repair of normal tissues compared poor capacity of cancer tissues
Destruction of small blood vessels on which growth of a tumour depends also
contributes to the effect and induction of an inflammatory response may -> destruction of
cells weakened by radiation
Increased sensitivity of cells in the presence of oxygen up to a critical level but above this
level (about that of normal atmospheric pressure) sensitivity does not increase
appreciably (hypoxic tissue has decreased radio sensitivity)
Radiosensitivity of a tissue is directly proportional to its mitotic activity and
inversely proportional to the degree of differentiation of its cells
Adverse effects:
1. Joint -> stiffness and loss of function -> physio
2. Subcutaneous fibrosis
3. Children -> premature fusion of growth plates
4. Irradiation induced sarcoma
5. Enteritis, diarrhoea, obstruction and bleeding
6. Cystitis and hepatitis
7. Scoliosis may develop therefore include both sides of vertebrae
8. Muscle atrophy and fibrosis
9. Erythema of skin and hyper pigmentation
10. Hair loss and skin flaking
11. Lymphoedema -> need to screen a strip of skin -> lymphatic drainage
Defiinitions
Rad (radiation absorbed dose) is a measure of the energy imparted to the matter by
ionising radiation per unit mass (1 Rad = 100 erg/gram (0.01j/kg)
Grays (Gr) = 1 joule of energy absorbed by a mass of 1kg (equivalent to 100rad)

13

`xzx|v g
o
o
o
o
o
o
o

Ewing's Sarcoma
Multiple Myeloma
Reticulum Cell Sarcoma
Hodgkin Lymphoma
Lymphosarcoma
Chloroma
Leukemia

15

Ewings Tumour
It is a primitive neuroectodermal tumour. It is the 3rd most common malignant 1ry bone tumor
(after MM & Osteosarcoma). Usually it occurs < 30 y, rapidly destroy the cortex and reaches the
soft tissues & metastasize. Usually the tumor is associated with fever and constitutional symptoms
Origin:

1st it was thought to be derived from endothelial element of BM

Now it is known to be a PRIMITIVE NEUROECTODERMAL TUMOR as
neuroblastoma
90% there is translocation at t(11:12)(q24;q12)

Incidence

5% of all 1ry bone sarcoma (3RD most common 1ry malignant)

90% occur between 10 - 30 y (peak at 2nd decade of life)
Rare before 5 & after 30y
Male : Female 3:2

Pathology

Site:
Long bones: FEMUR, tibia, fibula, metatarsals, humerus,
radius, ulna, metacarpals
o Flat bones: Scapula, pelvis, ribs, clavicle, & skull
o Irregular bones: Tarsus esp. The calcaneus & Vertebrae
Localization: DIAPHYSEAL
Macroscopically:
o Grey like BRAIN MATTER DD inspisated pus of OM /or (red
currant jelly if hgic)
o Occupy the medulla of the bone
o Growth usually exceeds nutrition supplies tissue lysis; hence the fever & the
Cysts filled necrotic debris (pus like) & the lytic appearance in PXR
o Necrotic lamellae in relation to the tumor is common feature
o Tumor extend via Haversian system to the surface leading to:
NBF in episodes ONION PEEL
Poorly demarcated and soft tissue extension common
Microscopically:
o HIGHLY CELLULAR tumor made of packed solid sheets of small cells
o cells are quite UNDIFFERENTIATED
o MONOTONOUSLY HOMOGENOUS with that conform to one shape round, or polyhedral
o Minimal intercellular substance
o NUCLEI: large / oval / HYPERCHROMATIC / fine regular chromatin / mitotic figures
o A ring of 7-8 cells around a central area of necrosis= "PSEUDOROSETTE" may form
o Within the centre of this rosette fibrils may be seen (=true rosette ccc of neuroblastoma)
o Hemorrhage and necrosis are typically present
o +ve PAS, SSS (sliver stains are of no help as it stains both collagen & reticulen fr)
o -ve reticulin stain
Spread:
o To distant sites via BLOOD and LYMPHATICS LN (? MULTI-CENTRIC from the onset)
o To other bones
o

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Clinically

1.

2.

3.

4.
5.

60% occur in long tubular bones (also pelvis ribs and scapula)
Considered a systemic disease
PAIN: ..........................................................................90%
o Vague, throbbing
o Starts intermittent long periods of remission (unlike any malignancy pain)
o Eventually, it becomes constant and severe
o worse at night
TENDERNESS of the lesion area
o spread of tumor via Haversian canals to outside to involve the soft tissues
including the nerves marked mass, all this before any PXR finding
o when Haversian vv are occluded sclerosis & lysis according to whether the vv
are completely or incompletely occluded
LUMP:
o Painful
o Hard
o Warm
o Diaphyseal
o Tender
o Smooth surface
o Ill-defined edges o Fusiform
o Slowly growing and its size y in episodes
METASTATIC deposits at presentation .........................(30%) Lung & LN most common
Patient may be ill and sometimes PYREXIAL ...............(20%)
o Fever may lead to mistaken diagnosis of osteomyelitis
o necrosis of the tissues; whose cells are closely related to pus cells
o Associated with y WBC, ESR
Pathological FRACTURE is rare ...................................(15%)
NEUROLOGICAL symptoms: .........................................(55% of pt spinal lesion)

6.
7.
8. CACHEXIA

Serology

Anemia
y ESR & WBC
y serum Alkaline Phosphatase
Vanyl Mandilic Acid (VMA) in urine to exclude neuroblastoma
Glycogen stains (PAS) to exclude Reticulum Cell Sarcoma
Electron Microscopy usually differentiate RCS
Biopsy is necessary
Cyto-genetics to determine the cell origin & translocation
PNET primitive neuro-ectodermal tumor t(11:22)(q24:q12)
Specific surface glycoprotein HBA71 & MIC2 are specific

X-Rays

ILL DEFINED, PERMEATIVE or MOTH EATEN lesions

DIAPHYSEAL
Usually LYTIC or mixed; but 40% may be sclerotic (patchy or streaky)
Continuous lamellated ONION PEEL periosteal reaction; deposition of one layer of NBF then

it is permeated by the tumor to produce another layer. Other periosteal reactions are also
present i.e. SUN RAY or CODMANS U
Rarefied area in medulla, but bone marrow infiltration is often not obvious on plain x-ray
Often the cortex is perforated
May look like osteomyelitis but diaphyseal

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MRI

MRI is essential to elucidate the soft tissue involvement

o T1 - z intensity compared to the high intensity of BM
o T2 - tumour is hyper intense compared to muscle

Bone Scan

increased uptake

Differential Diagnosis:

1.
2.
3.
4.
5.
6.
7.

Osteomyelitis is often the first diagnosis made ................ Metaphyseal

Neuroblastoma .................................................................. True rosettes,
Lymphoma (Reticulum cell sarcoma) .............................. Old, no glycogen, leukocytic AG
Osteosarcoma.................................................................... Metaphyseal
Chondrosarcoma ............................................................... calcifications
Metastatic Ca .................................................................... HBA 71 & MIC 2 ve
Metastatic embryonal rhabdomyosarcoma ....................... +ve actin, desmin, myoglobin /
electron microscopy shows cytoplasmic filaments and Z-band

Treatment

Best results with combined therapy (neoadjuvant wide en bloc radiation)

Wide surgical excision & limb salvage
Chemotherapy: 12 wks induction chemotherapy with VAC (vincristine, actinomycin D &
cyclophosphamide) are used preoperatively re-evaluate and restage surgical resection
Other chemotherapeutics: Doxorubicin, Isofosfamide, etoposide
Relatively radio sensitive
Radiotherapy associated y risk of pathological # & radiation sarcoma protected WB
is mandatory

Prognosis

70% 5 year survival (before multiagent chemotherapy was 5%)

Depend on:
o Site ........................................Distal is better
o Volume .................................Smaller is better (pelvic lesions are large)
o Metastasis at Dx....................Bad
o Age........................................the older the better
o Tumor necrosis to chemo .....>95 is good prognostic sign

14% of long term survivors develop secondary tumours and 1 - 2% are malignant (eg
leukaemia or osteosarcoma)
Young males and pelvic lesions -> worse prognosis.
If less than 10% viable tumour after chemotherapy =80% cure ;If not =20-30% cure

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Multiple Myeloma
Definition:

MM is a malignant tumor of PLASMA CELLS that causes widespread osteolytic bone damage
=plasma cell myeloma = plasmacytic myeloma = plasmacytoma = myelomatosis
Cytogenetic: recently common ch.abnormality at IG HEAVY CHAIN SWITCH REGION was found
tiology theoretical
Chronic inflammatory diseases
Herpes Virus 8 (HPV8)
PDF: Asbestos, Benzene, Pesticides, Radiation of atomic bomb
Epidemiology:

Commonest 1ry malignancy of bone > 40y (~ 40% of bone 10% of blood 1% of cancer)
May affect any bone with haematopoietic red marrow (spine, skull, ribs, sternum and pelvis)
Age 40-60 year
:=2:1
BLACK:white people also 2:1

Pathogenesis:

1.
2.
3.
4.
5.

Plasma cells are the most mature form of B-lymphocytes i.e. they secrete mature IgGs
Thats why it is ccc by OVERPRODUCTION OF MONOCLONAL IgG, IgA, and/or light chains
Sequelae depend on the site involved:
Skeletal:
MM produce OSTEOCLASTIC ACTIVATING FACTOR
bone is destroyed and replaced by tr pain & spinal cord compression & path#
hematologic
BM infitraltion by tr neutopenia, anemia, thrombocytopenia (PANCYTOPENIA)
Myeloid components interact clotting factors defective aggregation bleeding
Renal
Renal infiltration by plasma cells myeloma, light chain nephropathy, amyloidosis,
GLUMERULOSCLEROSIS hypercalcemia & hypercalcuria
Nervous:
Amyloid infiltration of nerves radiculopathy, cord compression, skeletal destruction
General
HYPER VISCOSITY sludging of the myeloid infiltrates in capillaries
Purpura, retinal hge, papilledema, coronary ischemia, CNS confusion or vertigo

Pathology:

Site:

FLAT BONES are the commonest (site of red bone marrow) skull/spine/sternum/ scapula/ribs

rare below the level of elbow and knee

Macroscopic
RED-GREY color the appearance & consistency of RASPBERRY CUSTARD
Surrounded by thin layer of cortical bone that show MULTIPLE INFARCTIONS
No reactive bone formation
Hge & cyst formation is common
Path# is frequent

19

Histology
Biopsy reveals SHEETS of densely packed plasma cells
ROUND or OVOID cells
1. ECCENTRIC NUCLEI typical CART WHEEL appearance = CLOCK FACE nuclei
2. CLUMPED NUCLEAR CHROMATIN beneath the nuclear membrane
3. BASOPHILIC CYTOPLASM ccc clear area HALO
4. The degree of cytological atypia of these cells has no prognostic value
OSTEOLYSIS occurs y cytokines released by the plasma cells osteoclastic resorption
HISTOLOGICAL CLASSIFICATION of MM:
1. Plasma myeloma
plasma cells
2. Myelocytoma
myeloblasts
3. erythroblastoma
Erythroblast
4. lymphocytoma
lympoblasts
Types:
1. Multiple myeloma
2. Solitary myeloma
3. Diffuse myeloma (myelomatosis)
4. chloroma: special type occurs in the skull; its cut section shows green color pigment
production that rapidly fades
Presentations

1- those mass destroying bones and replace BM

2- those excessive proteins
3- those impaired kidney function

1- Due to Mass:
1. PAIN

2.

3.

4.
5.
6.
7.

insidious bone pain starts in back, thorax, pelvis & precedes PXR findings
Duration of symptoms before dx ~ 9 mo
lytic effect on bone: Cortex is eroded from inside & Cancellous bone is melted away
Pathological FRACTURES
The tumor may remain silent till this event
thining and weakness of cortical bone
NEUROLOGICAL manifestation:
Compression fracture of a vertebra nerve root injury
Sciatica is a common & sometimes initial symptom
ANEMIA: extensive BM replacement
BLEEDING tendency: thrombocytopenia + interaction clotting factors by m proteins
CONSTITUTIONAL symptoms related to anaemia, thombocytopenia and renal failure
HYPERCALCEMIA: nausea, fatigue, thirst

2- Due to excess proteins:

Total serum protein may reach 10 gm/dl or more

Bacterial INFECTIONS are common because of a lack of normal Ig production.
HYPERVISCOSITY: FAHM, bruising, presthesia, sluggish mentation

3- Due to Renal failure: 2ry to

1.
2.
3.
4.
5.

Toxic Bence Jones ptn

Nephrocalcinosis
Amyloidosis
BJ ptn deposition in DCT internal hydronephrosis
Renal ischemia y viscosity

20
Investigations

CBC:
Pancytopenia (normocytic normochromic anemia)
Bleeding tendency (thrombocytopenia-abnormal coagulation)
y ESR (>100 mm/hr)
y Reticulocytes & Rouleaux formation
Chemistry:
o y Ca, AlkPh
o y creatinine
o y s.uric
o y LDH & 2microglobulins
o y serum proteins: >10gm/dl reversed A/G ratio (mainly in the globulin fraction)
S.Protein Electro-Phoresis (SPEP)& high resolution SPEP
o N/z alb
o N 1 & 2 &
o y Globulin = MONOCLONAL GAMMOPATHY (M-COMPONENT > 30G/L is mandatory to Dx)
o Accumulation of light chain proteins found in 20% and execreted in urine BJP
Immune fixation to determine the type of Ig:
60% IgG
20% IgA
1% IgD (IgM & IgE are rare)
Urine:
o y ca
o z sp.gravity (RF)
o Amyloid cast
o
o
o
o

BENCE JONES PROTEINS:

Light chain subunits of globulins

C

C precipitate 100

Detected by heating urine 50

cleared (or HCL)
If not detected collect 24hr urine UPEP or Immunoelectrophoresis
It is not diagnostic and found in leukaemia and secondaries

Radiology

PXR Skeletal survey is the MOST SENSITIVE; as Tc scan fail to show y uptake in 25%
The characteristic appearance of MM:
o Multiple, round, lytic, lesions
o Lesions are PUNCHED OUT, well defined, variable in size
o
o

NO PERIOSTEAL REACTION

Generalized demineralization
The characteristic appearance of single plasmacytoma:
o BUBBLY expansion of a single bone (rib or spine posterior arch)
o Associated SOFT TISSUE MASS
MRI
is useful for delineating spinal lesions & early cord compression
low T1 & high T2 (related to muscle); and it is enhanced the use of gadolinium
CT: Usually not needed in most of the patients; but it shows the extent of bony affection
Tc99m: COLD SPOTS (lesions are osteoclastic and not osteoblastic)

BM aspiration (sternal puncture)

BM biopsy (CT guided) accounts for more accurate dx than BM aspiration

y PLASMA CELLS: > 5% is suspicious 10% is established diagnosis

21
Treatment

Chemo + Radio Operation Drugs

Radiotherapy
o MM is Radio & chemosensitive, and reossification of tr defects may occur in months
o Radiotherapy is recommended for intractable bone pain & <50% affection of bone
o Initially there might be additional bone loss inflammation NWB for 4-6 weeks
Drugs: although MM is incurable many drugs were proposed to have a good effect
o Interferon 2b + prednisolone prolong disease free interval
o Bisphosphonates: z pain, recurrence, hypercalcaemia.
Chemotherapy
o Melphalan/Prednisone (MP) is the standard ttt for MM since 60s
o MP 4days 4wks MP these courses are repeated for a yearPalliative only
o Recent combination Vincristine, Adriamycin, Dexamthazone (VAD) especially for relapse
o Response to ttt & tumor burden are measured by M-component in serum or urine
(50% reduction is a good response)
Surgery
o Prophylactic IM nails for femoral, humeral deposits
o ORIF of other pathological fractures IM nails MMA
o spinal decompression for root compression or fracture
o if joint is affected arthroplasty is thought about

Prognosis

3% only show complete cure

Untreated, a patient will only survive an average of 6-12 mo usually from infection or hge
Keys for prognosis:
o Tumor burden
o Proliferation rate
Follow up of the treatment is by:
o Monoclonal Ig
o LDH & 2microglobulins z ttt
Poor prognosis :
o Tumor mass
o Hypercalcemia
o Bince Jones Proteins
o Renal failure
Multiple lesions - 5% 5YSR

distribution
common sites
predominant pattern
Morphology

MM
Symmetric
Skull, mandible, spine body (arch)
Osteolytic
Will defines
uniform

Secondaries
Asymmetrical
These sites are rare
Osteolytic or sclerotic
Ill defined
Variable sizes

22

Bone Lymphoma
(Non-Hodgkin's) reticulum cell sarcoma

=osteolymphoma = histiocytic lymphoma of bone

Definition

It is the 1ry malignancy of BM LYMPHOID CELLS. Although, 1ry lymphoma is potentially curable & 2ries
are fatal, both have the same cell type; but they are completely different in behavior & also in ttt.
Disseminated reticulum cell sarcoma is a generalized disease affecting bone & other lymphoid tissues
Criteria for diagnosis (definition):
HISTOLOGICALLY PROVED to be Lymphoma
NO OTHER Lymphoid affection
Solitary or multi bone lesion REGIONAL LN
tiology

VIRAL agents and immunosuppressives are accused

In IMMUNOSUPPRESSED pt after transplantations and AIDS

Epidemiology

3% of primary bone malignancy 5% of lymphomas

40-60 are common
Most intraosseous lesions are non-Hodgkin's lymphoma

Pathology:

Sites:
Long bones: FEMUR / humerus / tibia / fibula / radius / ulna
Flat bones: scapula (acromion) / ILIUM
Irregular bones: patella / VERTEBRAE / tarsal bones
Localization:
METAPHYSIO-DIAPHYSEAL lesion
Take at least the shaft of the bone
Macroscopic
NOTHING CHARACTERISTIC; PINKISH GREY mass
Begins IM and erodes bone from in to extend to soft tissues
Sometimes erosion is slow giving the chance of periosteum to
lay down shell of bone cortical expansion

G; BUT MORE PATHOLOG

LESS NECROSIS THAN EWIN
WING
ATHOLOGIICAL FRACTURE

Microscopic:

LAR
ARGE PLEOMORPHIC cells ( small uniform cells in Ewing) arranged
in SYNCYTIAL bands that are supported by primitive reticulum
Cell membrane is usually ILL DEFINED or IRREGULAR PSEUDOPODS

Cytoplasm:
o smooth / granular / basophilic
o cytoplasm : nuclear volume > 1:1
Nucleus:
o LAR
ARGE / HYPERCHROMATIC / REN
ENIFORM in mature tumor forms
o frankly lobulated in younger cells (may appear as a cluster of nuclei)
o High MITOTIC figures (> Ewing)
o COARSE IRR
RREGULAR CHR
HROMATIN (may look like Ewing=fine and regular)
o Nucleoli are evident
No intracellular glycogen ( Ewing)
+ve Argenophilia (SSS) reticulin fibrils (in Ewing it is +ve fine collagen fibrils)

23

Metastasis:
Late metastasis unlike Ewing
Mainly metastasize to LN > bone the opposite of the situation in Ewing
Both kill by lung metastasis
Staging:
SI
one bone lesion soft tissue mass
SII
Two lesions of the same side of the diaphragm
Or One lesion + regional LN
SIII
Two lesions on both sides of the diaphragm
SIV
BM or CNS affection
Clinically (slower than Ewing)

40-60

40 -50% occur around the knee

Good General condition (wt loss and night fever < 10%)
PAIN: ................................................60-100%
o Gradual onset more at night
o Dull aching
o Precedes the swelling
SWELLING:

Tender swelling, hard in consistency

Smooth surface, and illdefined border
LN enlargement of and affected region
Pathological FRACTURE
o More common and insidious than Ewing
HYPERCALCEMIA: especially in pediatric age group and associated poor prognosis
o
o
o

X-Rays

1. DOES NOT AFFECT LESS THAN OF THE BONE (extensive erosion). PXR often underestimate the

extent of the lesion

2. BONE DESTRUCTION:
Irregular, SMALL, MULTIPLE foci
MEDULLARY origin and eventually erodes the cortex in an irregular manner

Tumor behavior:
Lytic: Moth-eaten or Permeative ... 65%
Mixed: ............................................ 30%
Pure sclerotic: ................................ 5% (may be more common Ewing)
PATCHY MATRIX OPACIFICATIONS reactive NBF (indicate slow growth)
MOTTLED APPEARANCE: alternating areas of normal bone, lytic tumor & NBF
Little periosteal reaction y when cortex is eroded
3. SOFT TISSUE MASS:

Early in flat bones; but late in long bones

Large shadow out calcification
4. Pathological FRACTURES: not uncommon and occurs early in the disease
DDx

Ewings
Osteosarcoma
2ry

MM
MFH or fibrosarcoma: arise from the fibrous elements of the endosteum,
mainly IM extension, and saucerize the cortex late

24
Difference
1- Incidence:
2- Age:
3- Pathology:
A/ Site:
B/ Macro. :

Ewings sarcoma
More common
5-15

Reticulum cell sarcoma

Less than 1/2 as common as Ewings
40 - 60 y

Diaphyseal
Like brain matter, cheese or inspisated
pus (always mistaken osteomyelitis)
Necrotic lamellae

M&D. at least the bone is involved

Not ccc: malig.destructive mass involving shaft
& destroy cortex & extending to soft tissue
Less necrosis.

Medium size
Uniform
Contain glycogen
1 : 1 or 1 : 2
z
Rosette arrang. & in center by sss,
collagen fibrils are demonstrated

Large
Pleomorphic
No glycogen
y
y
Reticulin fibrils by special silver stain (sss)

BAD
severe
z
Much periosteal reaction
some bone destruction
Onion Peel
Most lethal bone sarcoma (95% )
It arises from BM very rapidly.
Metastasize early by all means
May be Radiosensitive but hazardous
to children

GOOD
Less severe
yy
Little periosteal react. But destructive &
expanding lesion
Mottled patchy matrix
30% can be cured by radio early Dx
It grows more slowly, metastasizes later
Excellent results even after LN metastasis
Radiosensitive more than other BM tumours

Cl Micro.:
1) Cells

2) Cytoplasm / nuc vol

3) Mitotic figures
4) Special silver stain
4- Clinically:
A) G. condit:
B) Pain:
C) Path. Fr.
5- X-ray;
6- Prognosis:

Investigations

CBC & blood smears to rule out leukaemia

y s.Ca
y ESR
y LDH
FLOW CYTOMETRY studies: to detect CD MARKERS of different lymphoma e.g. CD20 (Cluster of
Differentiation). It is simply a Ag-AB reaction (Ag is the CD marker in blood & the AB is
Fluorescent labeled) and the fluorescence is automatically detected by the flow device
CYTOGENETIC studies: to detect ch.abnormalities (common in lymphomas)
Biopsy: is mandatory for definite diagnosis
o Taken By:
Open biopsy
Core biopsy
FNAC only to retrieve cells and do Flowcytometric and cytogenetic studies
o Studies to be done:
Histopathology
Immunohistochemistry
Immunophenotyping
Cytogenetic study
BONE MARROW aspirates
CT
Abdominal exploration -> splenectomy -> staging

25
Treatment (mainly medical)

1.
2.
3.
4.
5.

Chemo & radiotherapy are used in conjunction in most cases

CHEMO: VAC-P (Vincristine, Adriamycin, Cyclophosphamide, Prednisone) for many cycles
RADIOTHERAPY: 40-60 Gy divided over several weeks (not used in children Growth R)
DRUGS: as anti-CD20 AB is used for B-cell lymphomas
BM TRANSPLANTATION: is reserved for refractory cases
SURGERY: ORIF for pathological #; amputations are not done now after modern trends in ttt

Prognosis

Lymphoma of bone has the best prognosis of all primary malignant bone tumors
30% cure correct early Dx
60% 5ysr ttt
Excellent results even after LN metastasis
It grows more slowly, metastasizes later
Radiosensitive more than other BM tumours

Other Lymphomas
Hodgkin
Lymphosarcoma
Follicular lymphoma
Lymphatic Leukemia
These tumors arise from
LN
Spleen
Intestinal Follicles
Liver
Skin
Thymus
In that order, and affect the BM reticulum as a part of generalized lymphoma, or may be 1ry

Hodgkin

26

Malignant granuloma of lymphoid reticulum

Affect bone as a part of generalized disease & boney lesions are the same as visceral ones
Pathology:

Commonest Boney sites to be affected:

Skull
LUMBAR spine
TheSecondCommon
Upper tibia
PELVIS
Lower femur
Upper FEMUR
Complex variety of cells: mainly inflammatory origin + reticulum hyperplasia
Epithelioid cells, histiocytes, lymphocytes, primitive reticulum cells.
REED STERNBERG cells:
Large, double nucleated cells
Chromatin is condensed on the nuclear membrane penciled delineation of nucleus
Nucleolus is large & pink staining
6 Histological types (The commonest is the GRANULOMATOUS type)
y eosinophilic granulocytes
Plasma cells, lymphocytes, fibrosis
Clinically:

20-30 y
1. LN:
The earliest manifestation
Discrete, smooth, soft rubbery
Painless, mobile
Late they become hard
2. CONSTITUTIONAL symptoms:
BEL EBSTEIN INTERMITTENT FEVER 15 d on & 15 d off

Anemia
z wt
3. ABDOMENAL examination
HSM LN
Ascites
4. BONY affection:
Late to occur after the disease is manifested in other tissues
Dull aching pain & tenderness
Cord compression
PXR
1.
2.
3.
4.

Lumbar spine:
Pelvis & Skull:
Ribs:
Long bones:

body collapse & typical appearance of IVORY VERTEBRAE

lytic areas surrounded by sclerosis
Lytic areas pathological # is pathognomonic
-Destruction + marked NBF (look like COM or unicameral bone cyst)
-Pure destruction as lymphosarcoma

27
Investigations:

1. CBC:
a. Anemia
b. Leucocytosis or leucopenia + relative lymphocytosis
2. CXR:
a. Hilar LN
b. Pulmonary Hodgkins
3. Biopsy: is the main parameter for accurate diagnosis
Treatment

RADIOSENSITIVE: Deep X Ray therapy z Pain & kill tumor

Amputation if failure to control the tumor

Lymphosarcoma
VERY RARE tumor of bone characterized by multiple bone affection if occur

Pathology:

Site: VERTEBRAE
Extend diffusely in the medulla ERODE THE CORTEX
Extend via Haversian canals subperiosteally to infiltrate soft tissues
NO NBF

Microscopic:

Diffuse LYMPHOCYTIC growth

RETICULAR stroma
NO MULTINUCLEATED CELLS

Clinically:

PXR:

Early PAIN
Vertebrae are commonly affected referred chest pain & LL pain
Pathological FRACTURE
Lytic PERMEATIVE lesions & NO NBF
Vertebral wedging

Treatment:

IRRADIATION z pain

Death in 3y

chloroma

It is a form of lymphatic or myeloid leukemia in tissues contain GREEN PIGMENT appear in cut sections
Children NEAR PUBERTY are the commonly affected
Rapidly FATAL in 5mo
Affects LN, spleen & bones; skull invade its structures esp.orbit exophthalmos & lid edema
Bones red BM also invaded by the tumor may extend to involve surrounding muscles &
tendons
Clinically, PXR, Microscopically: are the same as leukemia but:
Large ATYPICAL MONOCYTES
Mitotic figures
PRIMITIVE MYELOBLASTS
Hyperchromatic nuclei
Treatment: DXT

28

Leumkemia
Is a condition characterized by malignant production of immature lymphocytes (lymphoblastic) or
polymorphs (myeloblastic); resultant replacement of hemopoietic & lymphoid tissues
It occurs as acute or chronic forms ; the acute being common & predominant in children
Clinically:

1)
2)
3)
4)
5)
6)
7)

Fever
Anemia
z wt
HSM LN
Bone affection (50%): PAIN & palpable MASSES in superficial bones (e.g. Ulna)
Painful joints (synovial involvement)
Rib pathological FRACTURE

Pathologically:

Diffuse BM REPLACEMENT by leukemic infiltrate

Later ERODE CORTEX periosteal NBF
Types:
Lymphoblastic: commonly produce bone changes
Myeloblastic: short acute course less boney changes
Aleukemic: commonest around the knee, shoulder; despite its very rapid course
Investigations:

CBC:
Anemia
Thrombocytopenia
Lymphopenia or lymphocytosis
Immature WBC
PXR

1. DIFFUSE OSTEOPOROSIS

2.
3.
4.
5.

punctuate rarefied cortical lesions RAGGED CORTEX

TRANSVERSE RADIOLUCENT line on the metaphyseal side of the physis (zendochondral ossification)
LAMELLAR periosteal NBF
In chronic leukemia OSTEOSCLEROSIS (otherwise osteolysis)

Treatment:
IRRADIATION is effective for pain relief (eventually loses its effect)
MULTIAGENT CHEMOTHERAPY is the main ttt

Prognosis

Chronic leukemia have longer survival

Chronic leukemia in elders may remain inactive out ttt for years

Y|u g
o
o
o
o
o
o

Fibrous Dysplasia
Fibrous cortical defect & Non ossifying fibroma
Fibrosarcoma
MFH
BFH (Eosinophilic Granuloma)
Desmoplastic Fibroma

31

Fibrous Dysplasia
Non hereditary developmental disorder of bone forming mesenchyme a defective
osteoblastic differentiation & maturation; resultant abnormal growth. Unknown tiology
Incidence:

EQUAL sex and racial occurrence

REMAIN ASYMPTOMATIC: till 10y in Polyostotic & till 30y in Monostotic

Genetics:

FD is caused by a MUTATION IN (GNAS 1) gene that encodes for a protein Gs is present

almost in all cells and enables hormones to act on these cells producing an effect
In FD mutation occurs abnormal Gs cell acts as if the hormone is always present
The mutation effect depends on:
Time of occurrence during development
Type of embryonic cell affected
of tissues affected
When mutation occurs early Affect bones endocrinal glands

Pathogenesis

Normally mesenchymal stem cells (fibroblastic type) preosteogenic osteoblasts

In FD there is defective maturation y PREOSTEOGENIC cells & z osteoblasts are
abnormal:
Rounded
Less adherent : y versican, z osteopontin, z sialoprotein
Secretes more IL6 contributes to osteoclastic activation

Pathology:

NEA:

Medullary bone is replaced by fibrous tissue radiolucent on radiographs ccc

Ground Glass appearance
Scattered trabeculae of woven bone
There are fluid filled cysts embedded in fibrous matrix

Microscopic:

Types:

1.
2.
3.
4.
5.

Poorly oriented, C or S shaped woven trabeculae, that lack osteoblastic rimming

Small foci of cartilage
Fibrous stroma spindle cells oval nuclei & ill defined cell border
Areas of he accompanied by osteoclastic Giant cells
Pathological fr on previous lesions reactive NBF osteoblastic rimming and
periosteal reaction
Monostotic
Polyostotic
Craniofacial
Cherubism
McCune Albright $

32
Monostotic:

70%
Sites: Rib (28%), Femur (23%), Tibia, Craniofacial (10-25%), Humerus
CP:
Almost 10-30 y
Pain
Pathological #
Less severe deformity than polyostotic

Polyostotic:

30%
Sites: Femur (90%), Tibia (80%), Pelvis (70%), Ribs (60%), Skull (50%), UL,
Lumbar spine, Clavicle, Cervical spine
May be Monomelic, Unilateral, Bilateral
CP:
Pain, limp, Pathological #, usually before age of 10
Deformity and LLD
Femoral coxa vara + upper femoral bowing Shepherds Crook Deformity

Craniofacial:

May be part of monostotic, or polyostotic, or occur as an isolated form as in here

In isolated form no other skeletal affection
Commonest sites: Frontal sphenoid maxilla ethmoid
Less common: occiput and temporal
CP:
Cranial asymmetry & facial deformity
Orbital affection: exophthalmos & blindness
Sphenoid & temporal: vestibular manifestation, vertigo, tinnitus, hearing loss
Cribriform plate: anosmia, hyposmia

Cherubism:

AD
Regress after adolescence
Broad protruded jaws; both maxilla & mandible are symmetrically affected

McCune Albright $

Polyostotic
Precocious Puberty
Caf au-lait spots

Clinically:

Wide spectrum; from monostotic asymptomatic cases to polyostotic markedly deformed pts.
70% of patients presents pain
Deformities by the age of 10y
Pathological fractures
Special presentation: according to type; e.g. blindness, hearing lossetc

PXR

Long bones:
Metaphyseal or diaphyseal lucent lesions (may extend to epiphysis after closure)
Lesions matrix is homogenous and classically have the Ground Glass appearance
Endosteal scalloping Bone expansion
Femoral coxa vara + upper femoral bowing Shepherds Crook Deformity
Bone may show calcifications & endochondral NBF, but absent periosteal reaction
Premature closure of physis may occur adult dwarfism

33

DDx

Skull and facial bones:

Frontal bone > sphenoid and both are affected sclerotic base
Obliteration of sphenoid and frontal sinuses
Lucent or sclerotic lesions in skull or facial bones may be seen
Pelvis and ribs:
Lytic lesions Ground Glass appearance
Cystic lesions are common
Protrusio acetabuli is common ?? Kohlers line
Enchondromatosis
Eosinophilic Granuloma
Giant cell tumor
Hemangioma
Hyperpauathyroidism
Neurofibromatosis
Pagets

C o m p l i c a t io n s :

1. Malignant transformation:
0.4-1%
May take several years to develop
Commonest are: skull & facial lesions in monostotic / femur & facial in polyostotic
Osteosarcoma, fibrosarcoma are ! commonest transformation (chondrosarcoma is less)
PXR:
Rapid y in size
Change of the sclerotic lesion to be lytic
Clinically:
y pain
y size of soft tissue mass
2. Metabolic changes:
Hypophosphatemic rickets and osteomalacia occur FD
May be because FD secretes phophaturic hormone

T r e a t m e n t:

Monostotic lesions are usually asymptomatic and needs no treatment

Indications for excision:
1. Severe or progressive deformity
2. Nonunion of a fracture
3. Femoral shaft fracture in an adult
4. Persistent pain
Intralesional excision and autogenous BG unsatisfactory results
Internal fixation has no effect on the disease but fix the weak bone
Multiple osteotomies to correct the deformities + IF
In expendable bones enbloc excision

34

Fibrous Cortical Defect

& Non-Ossifying Fibroma
Incidence

20% of benign bone tumours

Male more than Female

Clinically

Usually an incidental finding in children

Most heal spontaneously
Larger ones may -> pathological fracture (common presentation)

X-Rays

Lucent lesion in the cortex of a long bone

metaphyseal
eccentric
Margin well defined, sometimes scalloped and often sclerosed

Histology:

whorled fibrous tissue, foam cells

occasionally, small elongated giant cells

Differential Diagnosis:

1.
2.
3.
4.
5.

malignant fibrous histiocytoma

eosinophilic granuloma
osteosarcoma
histiocytic lymphoma
pyogenic osteomyelitis

Treatment

Most spontaneously resolve or move to the diaphysis of the bone with growth
Pathological fractures:
usually heal with a normal amount of callus, but resolution of the fibroma may or may not
occur
intracapsular curettage is usually sufficient to promote healing of lesion, however, the
defect may be supplemented with bone grafts +/- stabilisation

Jaffe-Campanacci syndrome:
multiple FCDs
cafe-au-lait spots
mental retardation
hypogonadism
ocular & cardiovascular abnormalities.

35

Fibrosarcoma
Rare tumor that may arise in the medulla (central), or on surface
(periosteal)
Differentiation is difficult bet. LG fibrosarcoma & the aggressive
benign tumors (desmoplastic fibroma & periosteal desmoid)
Also the diff. bet. HG fibrosarcoma and osteosarcoma & MFH
Incidence
2% primary malignant bone tumours
Middle age
But it occurs in any age and sex
tiolo
gy
og
no definite cause
Mutations and translocations are suspected
2ry fibrosarcomas (on top of other pathologies):
VonRecklinghausen $
FD
COM
Bone infarcts
Pagets
Irradiated bone
In association implants used for fixation
Types:
Central (medullary)
Peripheral (periosteal)
Difference
1- Incidence:
2- Behavior
3- Pathology:
A/ Site:
B/ Macro. :

Cl Micro.:
1) Cells
2) Whorly appearance
3) Bone Trabeculae
4- Clinically:
A) Principle complaint
B) Pain:
C) Path. Fr.
D) Metastasis
5- X-ray:

Central
10% of 1ry bone malignancy
Early destructive

Peripheral
Less frequent
May encircle the whole bone (out NBF)

Metaphyseal

Origin from the fibrous layer of periosteum

Extracortical & firmly anchored to bone
Pale color (sometimes white and glistening)

Whitish grey (predominant collagen)

LG is more white
HG is fast invader
Early cortical invasion
Saucer shaped excavations pressure necrosis
Vascular
Less vascular
Some NBF
No NBF (if bone formation Parosteal)
No capsule
Pseudo-capsule compressed surroundings
Both show areas of hge, necrosis, and cystic formation
Same as than of fibrosarcoma of the skin
Fusiform cells or spindle cells
Same
Very evident fibrous whorls
Less evident
Wave through the tr substance
Less bone formation
Pain
severe
Common
Late
Central lytic lesion
Cortex is penetrated
Periosteal NBF is evident
Soft tissue mass after cortical invasion

Mass: mobile, lower femoral, steadily y size

Late after mass by years
Less common
Late
Faintly outlined soft tissue shadow out NBF
Saucer shape depression
Periosteal reaction at the periphery
Soft tissue shadow is very important

36

Histopathological Grading:
Collagen
Spindle Fibroblasts
Nucleus
Chromatin
Mitosis
Nucleoli

Grade I:
Abundant (Thinner Than Desmoid Tumor)

Grade II:
Thin Collagen

Hyperchromatic Nuclei
Coarse Uneven
Few
May Be Prominent

Large Plump Irregular

Clumped
Many
Large And Evident

Differential Diagnosis:
Neoplasm

Non Neoplasm

Metastatic carcinoma
multiple myeloma
MFH

paget
FCD NOF
Reticulo-endotheliosis

Prognosis:
Fibrosarcoma recur rather than metastasize
Like parosteal OS & chondrosarcoma; tend to change their cytological ccc after surgery
10 YSR is 30% in primary FS; and < 10% in secondary FS
Treatment
LG Stage I limb salvaging excision with wide margin
HG StageII radical or wide margin adjuvant chemoradiotherapy
Classically considered radio-resistant
May however be useful as adjuvant to surgery and chemotherapy
Useful for irradiation of lung secondary deposits not accessible to surgery

37

Malignant Fibrous Histiocytoma

Incidence
5% of primary malignant bone tumours
Age: > 30 (often > 50)
Male > Female
tiology and histiognesis:
80% are primary
20% 2ry to: Pagets, Infarcts, GCT, Fibrous dysplasia, Enchondroma, Radiation, ORIF
Cell of origin (controversial): histiocytic cell; fibroblasts; multipotent mesenchymal cell
Classification
1. Myxoid
2. Non Myxoid
Clinically
Present with pain, swelling
15% present with a pathological fracture
2ry to: Pagets, fibrous dysplasia, long standing osteomyelitis or irradiated bone
40% occur around the knee
Metastasises to the lung, and other bones via the blood
X-Rays
Usually metaphyseal
around knee
bone often mottled or moth eaten with extension into soft tissue
Osteolytic lesion may be surrounded by reactive bone
Destructive appearance radiologically
Usually little periosteal reaction
Pathology
Discrete greyish white rubbery tumour
Irregular bony margins
Composed of a fibroblasts, myofibroblasts & large plump foamy histiocytes
The fibrous component is in a "storeiform pattern" (radiating)
Differential Diagnosis:
1. Metastatic carcinoma
Treatment
Radical excision / Amputation or radiotherapy if not amenable to resection
No randomised studies but reports of increased survival with neo adjuvant chemotherapy
(doxorubicin, vincristine, methotrexate, T10)
Prognosis
Better in young
If initial procedure a wide or radical excision = more than 80% 4yr survival

38

Benign Fibrous Histiocytosis

It is the benign spectrum of Langerhan's cell histiocytosis, characterized by single or multiple
skeletal lesions in young age group
Terminology:

Eosinophilic Granuloma (EG)

Histiocytosis X (the old name) = Langerhans cells histiocytosis

Xanthofibroma = Fibroxanthoma
Sub-types:
1. Hand Schuller Christian disease
>3y
Triad of skull lesions & exophthalmos & diabetes insipidus
Occasionally: wide spread visceral involvement (liver, pituitary)
cranial lesions are always present in this disease
2. Letterer-Siwe disease
<3y
liver, spleen, skin, CNS involvement
Rapidly fatal
look for recurrent bacteremia, diffuse LN & skin lesions
Aetiology
Solitary EG & HSCD are non-neoplastic reaction of RES
histiocytes to unknown stimulus
Letterer-Siwe disease is a lymphomatous proliferation of less differentiated RES histiocytes
histiocytes
There is accumulation of metabolic products of lipids in RES StoredIn
inflammatory granulomatous response occurs.
Incidence
1 - 5% benign bone tumours, usually solitary
80% < 10 y (usually 5-10 y)
:= 3:2
Pathology
Sites: affect any bone (NB; distal to elbow and knee is rare)
skull (common), mandible, spine, ribs, pelvis, and diaphyses
femur is the most frequently involved long bone
Mainly diaphyseal ( NOF)
NEA
Glistening reddish tissue with flecks of yellow
Mic
Pale / foamy / lipid filled histiocytes
Mixed eosinophils, plasma cells, PNL & giant cells + Back ground of spindle fibroblasts
Langerhan's cells: (Langerhans histiocytes)
Grooved or coffee bean shaped nucleus
Abundant pale staining cytoplasm
Well defined cytoplasmic borders
EM: racquet-shaped cytoplasmic inclusions (Birbeck granule) /
Periphery contain bone spicules
there should be no evidence of cartilage or bone matrix

39

Clinically
Mostly asymptomatic
Pain & tenderness
Palpable mass at the lesion site
Pathological # (Rarely)
Lesions of the skull
Elicits a more lytic & scalloped appearance
May be associated with a palpable soft tissue mass
Pelvic lesion: subtle hip pain y by movement
X-Rays
Diaphyseal ( NOF metaphyseal)
Oval Mottled lytic defect usually no sclerotic rim
Starts intramedullary and erodes the endosteum as it enlarges
Eventually it erodes the cortex bone expansion & cortical tunneling
Trabeculation: May appear loculated due to sparing of large trabeculae
Periosteal lamellated NBF
Sometimes soft tissue mass is encountered
Flat bones and ribs
lesions appear punched out
Cortical scalloping may occur
Spinal lesions:
Collapse (vertebra plana)
Scoliosis may occur
More in dorsal spine (body or arch) and more in children
Sometimes no localised bone lesion but generalised osteoporosis
99m
Tc
Usually hot (but sometimes cold)
Differential Diagnosis:
1. Ewings: soft tissue mass arising from the tumor lesion
2. Osteomyelitis
3. ABC
4. FD
5. Lymphoma
6. Leukaemia
Treatment
Usually heal spontaneously and need no ttt except when it is large and symptomatic
Intralesional excision and grafting disappointing recurrence more extensive lesions
Wide local excision + BG is recommended
Steroids is another option and tends to result in rapid resolution
Radiotherapy for aggressive or inaccessible lesions (500-1000 rads)
Prognosis
Letterer Siwe disease worst prognosis often fatal in infancy
Hand Schuller Christian : excellent prognosis if there is no extra-osseous disease
Skeletal lesions: only do not death
Soft tissue involvement & rapid course & young age = worse prognosis
Liver involvement 50% die
Lung involvement usually not fatal
Anaemia increased mortality and indicates poor prognosis

40

Cortical Desmoid
(Desmoplastic Fibroma)
RARE tumor of bone the may present as an intramedullary or periosteal tumor. And histologically

is identical to extraosseous desmoid tumor. It is a mere asymptomatic irregularity in the

posteromedial aspect of the distal femoral metaphysis. It may be a reaction to muscle stress
exerted by the adductor magnus.

Incidence:
< 30Y (70%) .............................................................................................. (unlike fibrosarc)
Pathology:
Gross:

Dense white and WHORLED appearance of fibrous collagen

Round LOBULATED margins
Recurrent lesions erode and extend to soft tissues
Microscopic
MONOTONOUS arrangement of collagen fibers & fibroblasts
COLLAGEN bundles are thick and wavy and > 10 ............................. (unlike FD)
FIBROBLASTS OVAL NUCLEI FINE CHROMATIN & TYPICAL mitosis........ (unlike fibrosarc)
No multinucleated giant cells or foam cells ...................................... (unlike NOF)

Clinically:
if any, PAIN
Painless SWELLING
Occasionally PATHOLOGICAL #
PXR:

The lesion is best seen in oblique view ER 20-45

5CM AGGRESSIVE TRABECULATED LYTIC lesion
Endosteal EROSION & cortical expansion
SCLEROTIC margin FD
Aggressive wide zone of transition fibrosarcoma

Treatment:
It is more aggressive than other fibrous lesions and Enneking recommended WIDE EXCISION as
the method of choice
If wide excision gonna make a big deal of functional deficit intralesional CURETTAGE + BG
adjuvants:
Phenol
Cryotherapy
NB: GARDNER SYNDROME: Desmoid tumors + osteomas + epidermal cysts + colonic polypi

41

bxzx|v g
o Osteosarcoma
o Osteoid Osteoma
o Osteoblastoma

42

Osteosarcoma
Defiin
niittion:
1ry sarcoma of bone MALIGNANT OSTEOID FORMATION arising from MESENCHYMAL BONE FORMING
cells
It is the commonest 1ry bone sarcoma
Classificatiio
on:
Osteosarcoma
Intracortical

Extra-Skeletal

Conventional

Osteoblastic

Chondroblastic

IM

Telangiectatic

Fibroblastic

Multifocal

Small cell

LG IM

Parosteal

Juxta-cortical
(surface)
Periosteal

Secondary

HG surface

Mixed

I- Intra-Medullary Osteosarcomas

1- Conventional Osteosarcoma
Inciid
dence:
2ND MOST COMMON PRIMARY malignancy of bone behind MM (20% of all 1ry sarcomas)

~ 20% of primary malignancies of bone

~ 1-3/106/Y
ADOLESCENCE; if occur > 40y it is usually 2ry to:
Paget's disease
Radiation
No significant racial differences
Genetic Factors:
Rarely play a role, although osteosarcoma is common in pts hereditary retinoblastoma
ONCOGENES in osteosarcoma- Retinoblastoma gene and P53
Cliin
niic
callly
y:
PAIN is the most common complaint:

HG OS progressive pain
LG OS painless swelling
initially may improve with conservative measures and activity modifications
eventually becomes severe if the diagnosis is delayed
Night pain is very important clue (only 25% of patients)
Tender, soft tissue SWELLING
Patients frequently are MISDIAGNOSED with a more common problem at the initial visit
The average DELAY from onset of symptoms to the correct Dx 15 wk :
failure to obtain PXR at the initial visit
failure to repeat the PXR when a patient's symptoms persisted or worsened

43

Pa
Pathological features
Site:
METAPHYSEAL (90%)
Commonest sites: DISTAL FEMUR, PROX TIBIA & PROX HUMERUS

Macroscopic:
GRAYISH FLESHY mass eroding the cortex & the periosteum.
Areas of HEMORRHAGE, NECROSIS & CYSTIC degeneration
May FUNGATE outside the bone & involve the soft tissues

(usually by time of presentation)

NEVER PERFORATE the skin if reached it; only stretches it

As the neoplasm permeates the cortex, the periosteum may be

elevated NBF "CODMAN'S U ". Codman's U is a bit of a misnomer, it is not a
complete U (growth is too fast for the periosteum, so it responds thin shells of NBF)
only the edges will lay down NBF ossification is seen tangentially on PXR as a
small angle bone surface, but not a complete U
As growth continue the (SHARPEY'S FIBERS) that connect the periosteum to bone are
stretched out to bone when NBF is laid down around these fibers and ossify
"S
SUNBURST" or "H
HAIR-ON-END" patterns of periosteal reaction
Wide extension within the marrow cavity
Rarely, SKIP LESIONS within the medullary canal is seen
Microscopic: (Key ward for definitive Dx is MALIGNANT OSTEOID MATRIX)
Spindle Cells:
HAPHAZARDLY arranged
PLEOMORPHIC
HYPERCHROMATIC and irregular nuclei
MITOTIC figures, often atypical, but usually numerous
MALIGNANT OSTEOID MATRIX: lace-like eosinophilic matrix deposited among these cells
Both malignant and benign OSTEOBLAST-LIKE GIANT CELLS (confuse GCT)
HISTOLOGIC SUBCLASSIFICATION: according to the predominance of one tissue type

Osteoblastic Osteosarcoma:
1. Malignant osteoid (MO) prevails
2. Arranged as a delicate meshwork or broader confluent masses
3. Calcification of the matrix is variable .
Chondroblastic Osteosarcoma:
1. Malignant cartilage production
2. Still there must be malignant osteoid matrix present to Dx
fibroblastic osteosarcoma
1. Large areas of fibroblasts arranged in intersecting fascicles.
2. Such areas are indistinguishable from fibrosarcomas
3. Thorough sampling is necessary to identify the MO component.

44

Radiological features
METAPHYSEAL lesion (~ 10% are primarily diaphyseal & < 1 % epiphyseal)
MEDULLARY lesion radiodensity & radiolucency
PERMEATIVE invasion poorly defined borders & wide zone of transition
Cortical DESTRUCTION
Periosteal elevation & reaction SUNRAY , HAIR-ON-END, CODMANS
Extra-osseous EXTENSION, and soft-tissue CALCIFICATION
RADIOLOGICAL CLASSIFICATION:

1) Sclerotic osteosarcomas............... (30%)

2) Osteolytic osteosarcomas ............ (25%)
3) Mixed pattern .............................. (45%)
There is no prognostic value for the radiographic matrix
Errors in Dx often occur pure osteolytic osteosarcoma.
Mixed Matrix Pattern

2- Telangiectatic osteosarcoma
RARE - 5% of all osteosarcomas
AGGRESSIVE
Presents with PATHOLOGICAL FRACTURE
May look like ANEURYSMAL BONE CYST

Radiology

Often PERMEATIVE osteolytic cortex erosion

Sometimes has BALLOONED appearance
expansion
PERIOSTEAL reaction & Codman's U

MRI
T1 - high intensity with fluid-fluid levels.
Pathology

site: same
Gross
Multi-cystic "BAG OF BLOOD"
May look like ABC
Mic
Large blood filled spaces and thin septa; low power (DDx ABC)
Within the septa there is MO + pleomorphic malignant cells; high power
Prognosis

Poor

45

3- Small-Cell Osteosarcoma
Small cell osteosarcoma, another RARE variant
It is HG LESION that consists of small blue cells that may resemble Ewing or lymphoma.
Cytogenetic and immunohistochemistry studies sometimes are needed to differentiate

Histologically:

In small-cell osteosarcoma the neoplastic cells are round rather than spindle-shaped
Consists of NESTS & SHEETS of blue cells separated by fibrous septa .......... (similar to Ewing)
Cells:
Small round blue cells
Well defined border
Distinct rim of cytoplasm
Round nuclei Fine chromatin
Occasionally, transition to spindle cells is noted
LACE-LIKE OSTEOID MATRIX often surrounds small nests of cells confirms the diagnosis if
present. It may present in only a small quantity, can be difficult to differentiate from the
fibrin-like material that may be present in Ewing sarcoma.

4- Low-Grade Intramedullary Osteosarcoma

RARE type characterized by an INDOLENT course relatively BENIGN features on PXR

Mistaken histologically & on PXR for an osteoblastoma or fibrous dysplasia

Pathology:
As the name implies, it is located in an intramedullary location

Only erodes through the cortex very late

M ic

Slightly atypical spindle cells producing slightly irregular MO trabeculae

46

II- Juxta-Cortical Osteosarcomas

1-Parosteal Osteosarcoma
It is a distinct variant of osteosarcoma that arises from the cortical bone characterized by
SLOW growth and LATE METASTASIS

Incidence:

~ 4%
OLDER age group
Has a BETTER OVERALL PROGNOSIS than osteosarcoma (long-term survival rate ~ 75-85%)

Pathology:

Site:
Arise form the CORTEX of DISTAL FEMUR ..............(most common location)
Characteristically, ATTACHED TO POSTERIOR FEMORAL ASPECT
Prox humerus & tibia .......................................(next most frequent sites)
NEA
Tends to form a PROTUBERANT LOBULATED ossified mass
Other areas may INFILTRATE into the adjacent soft tissues
The tumour usually ENCIRCLES (partially or completely) the bone
covered in part by a CARTILAGINOUS CAP (DD. Osteochondroma bone medulla is
not continuous that of the neoplasm)
Microscopically:
It is regarded as a Mesenchymoma i.e. it contains all mesenchymal tissues:
Bone
Cartilage (bluish white)
Fibrous tissue
Even muscles present in peripheral parts (after it invades the surroundings)
Classically LG lesion:
irregular MO trabeculae surrounded by a FIBROBLASTIC spindle cell
There may be foci of ATYPICAL CHONDROID differentiation
HG lesions: y the chance for IM involvement

Clinically:

Usually present as a mass pain.

Radiological features
LARGE. DENSE. LOBULATED mass
Broadly ATTACHED to the underlying bone WITHOUT INVOLVEMENT OF THE MEDULLARY CANAL
The tumour may ENCIRCLE the entire bone.
IM EXTENSION is difficult to determine on PXR & accurately detected CT

DD: Commonly mistaken as osteochondroma, myositis ossificans, or conventional OS

47

2-Periosteal Osteosarcoma
Periosteal osteosarcoma is a rare INTERMEDIATE-grade malignancy that arises on bone SURFACE
It occurs in a slightly OLDER and broader age group.

Pathology:

Site:
DIAPHYSES of the femur and tibia

NEA
It projects into the adjacent soft tissues as a well-CIRCUMSCRIBED LOBULATED mass
Histologically:
Intermediate-grade chondroblastic OS
The tumour reveals a DOMINANT CHONDROID LOBULES
May contain markedly ATYPICAL CHONDROCYTES.
At the periphery of the lobule the SPINDLE-CELL component produced intercellular MO
Areas of MO and chondroid matrix can be seen to INFILTRATE the cortex

Radiologically

Small. RADIOLUCENT lesion with some evidence of BONE SPICULATION

The cortex is characteristically INTACT with SCOOPED-OUT appearance and a CODMAN'S U

3-High-Grade Surface Osteosarcoma

High-grade surface osteosarcoma is THE LEAST COMMON TYPE of osteosarcoma
As the name implies, it is an AGGRESSIVE tumour arising on the outer aspect of the cortex.

Histologically:

Like conventional OS: HG tumour y cellularity, mitosis & nuclear pleomorphism

Medullary invasion is common at the time of presentation ( Parosteal OS)

Radiologically

invasive lesion with ill-defined borders

48

III- Secondary Osteosarcomas

Secondary osteosarcomas occur at the site of another disease process.
They rarely occur in young age & 50% of the 2ry osteosarcomas > 50 y
The most common factors associated 2ry osteosarcomas include Paget & previous radiation
PAGETS SARCOMA
Incidence
Age: 50-70y
Male : Female 2:1
Occurs in advanced POLYOSTOTIC disease 10%
Affects 1% of all patients with Pagets
Pathology
PELVIS is the most common site
50% or more are osteosarcomas
25% fibrosarcomas
Remainder are chondrosarcomas or anaplastic tumours
Prognosis
Poor - < 50% 1 year survival
RADIATION SARCOMA
Occurs ~ 1 % of patients who have been treated with > 2500 cGy
Can occur in unusual sites e.g. skull / spine / clavicle / ribs / scapula / pelvis
Criteria for diagnosis:
1. Evident BENIGN INITIAL lesion (both histologically & radiologically)
2. Must be identified HISTOLOGICALLY & DIFFER from original pathology
3. 2ry OS must arise in RADIATION ZONE
4. LATENT PERIOD must pass (at least 5 y) before appearance of the 2ry lesion
Earlier appearance in younger patients
Prognosis
Cumulative: 5YSR = 17%
OTHER CONDITIONS that have been reported to be associated 2ry OS include:
1. Fibrous dysplasia
2. Bone infarcts
3. Osteochondromas
4. Chronic osteomyelitis.

49

IV- Multifocal Osteosarcoma

Osteosarcomatosis
Multiple sclerotic osteosarcoma is HG OS characterized by multiple intraosseous foci of
osteosarcoma at the time of presentation
Previously osteosarcomatosis was considered to be MULTICENTRIC PRIMARY NEOPLASIA
More recently, it is suggested that it is RAPIDLY PROGRESSIVE METASTATIC DISEASE.

Incidence:
UNCOMMON ~ 3-4% of osteosarcoma

However, it was found at autopsy in the rate of 48%

More common in SKELETALLY IMMATURE patients (but it occurs also in the old age group)

Pathology

In young pts: SPEEDY, SYMMETRIC, SCLEROTIC lesions

In old pts: it is fewer, asymmetric sclerotic lesions
Radiologically

1. The dominant lesion:

PERMEATIVE aggressive behavior ill-defined margins
Usually contain Cloud-Like Osteoid, but also, purely LYTIC lesions may be seen
Cortical DISRUPTION, aggressive periosteal REACTION, & soft-tissue EXTENSION
2. The secondary foci:
Smaller, more SCLEROTIC, and better DEFINED
Lack cortical destruction or periosteal reaction
Prognosis

Very poor even intensive chemotherapy

Patients die in 1y

V- Intra-CorticalOsteosarcoma
This is the RAREST osteosarcoma, and the term applies to the lesion arises in the cortex
It was 1st described by Jaffee in 1960 in a report of 2 cases, & through 1991 only 9 cases
Pathology:

Site
INTRACORTICAL in the FEMUR or TIBIA
Measures < 4 cm in diameter

Microscopically:
SCLEROSING variant of osteosarcoma (MINERALIZED MO)
May contain small FOCI of chondrosarcoma or fibrosarcoma.
Radiologically:

Although it is difficult to make generalizations on the basis of such a small of cases;

Lesion shows GEOGRAPHIC bone lysis + dense areas of osteoid formation (G IA Ludwick)
Well DEFINED edge THICKENING of the surrounding cortex
Medullary invasion is only rarely reported.

Prognosis

High rate of METASTASIS (29%) may be sufficient to justify adjuvant chemotherapy

50

VI- Extra-SkeletalOsteosarcoma
=Soft-tissue OS is RARE 1.2% of all soft-tissue sarcomas & 4% of all osteosarcomas
>
Clinically:

Slowly growing PAINFUL MASS

History of trauma................................................ (15%)
Pathology:

Sites:
Deep soft tissues of the THIGH ................. (45%)
UPPER extremity ....................................... (20%)
RETROPERITONEAL ...................................... (15%)
Osteosarcomas are usually large ....................... (~10cm)
Histologically:
Variable amounts of MO
Cartilage, fibrosarcoma, MFH, and Schwannoma all are seen (MESENCHYMOMA)

Radiologic

Large soft-tissue masses focal to massive areas of mineralization & z osseous involvement
Pseudo capsule may also be apparent

Scintigraphy

y uptake in both primary and metastatic foci

Treatment:

Amputation or wide surgical resection with neoadjuvant chemotherapy or radiation therapy

Prognosis:

Tumour size is the most important prognostic factor (> 5 cm having a bad outcome)
Despite aggressive therapy, overall prognosis is poor (Death >60%)
Metastases are frequent to Lung, LN, bone

51

Differential Diagnosis
Radiological
Osteosarcoma
Osteolytic

Osteosclerotic

Fibrosarcoma
MFH
ABC
GCT
Brodies abscess

Mixed

Osteoid osteoma
Stress fracture
Osteoblastoma
Ewing

Osteoid osteoma
Stress fracture
Osteoblastoma
OM

Pathological
Osteogenic

Low grade Intramedullary

1.Osteoid osteoma
2.Osteoblastoma
3.S tress fracture
4. Healing callus

1.NOF
2.Fibrous dysplasia
3.Osteoblastoma
4.Chondomyxoid fibroma

Chondrogenic

Small cell OS

1.Chondrosarcoma
2.Enchondroma
3.Chondroblastoma

1.Ewing sarcoma
2.Plasma cell tumour
ParostealOS

Fibrogenic

1.Myositis ossificans
2.Osteochondroma

1.Fibrosacoma
2.MFH
3. Fibrous dysplasia
4. Chondomyxoid fibroma

Periosteal OS:

Myositis ossificans

Giant cell type

Giant cell tumour

Osteosarcoma
Intracortical

Extra-Skeletal

Conventional

Osteoblastic

Chondroblastic

IM

Telangiectatic

Fibroblastic

Mixed

Multifocal

Small cell

LG IM

Parosteal

Juxta-cortical
(surface)
Periosteal

Secondary

HG surface

52

Prognosis of Osteosarcoma
Between 70 - 90% of osteosarcomas in the limbs can be treated with limb-sparing surgery
and chemotherapy so that amputation is not necessary.
When osteosarcoma affects one limb, the LONG-TERM SURVIVAL RATE (LTSR) is 60 - 75%
The prognosis will depend on :
1. Spread
5. Response to chemotherapy
2. Site
6. Type of osteosarcoma
3. Size
7. Serum markers
4. Surgery
8. General health
1- Tumour Spread
Patients localized disease better prognosis ........................5YSR 70%
20% of pts have lung metastases at diagnosis ...........................5YSR 40%
The prognosis for metastatic disease depends on:
Site

Surgical resectability of the metastatic disease.

2- Site Locatiio
on
Axial skeleton primary .............................................................y risk of progression & death
Pelvic osteosarcomas ~ 10% of all osteosarcomas....................5 YSR 20-40%
3-Surgery
Resectability of the tumor is the MOST IMPORTANT PROGNOSTIC factor because this tumor is
very resistant to radiation therapy.
Complete surgical ablation of 1ry & 2ries + neoadj. ................good LTSR
Unilateral lung metastases & fewer LN is better than bilateral, and many LN
4- Type of osteosarcoma in relation to conventional OS
Multifocal sclerosing OS...........................................................Very bad
2ry OS........................................................................................Poor prognosis.
small-cell OS .............................................................................Poor
Telangiectatic OS .....................................................................was worse & now the same
HG surface OS...........................................................................AS conventional OS
Intramedullary LG OS...............................................................Good prognosis.
Periosteal osteosarcoma.............................................................Better than conventional
Parosteal osteosarcoma..............................................................Best prognosis
Intracortical OS prognosis.........................................................Unclear because of its rarity
Multicentric osteosarcoma prognosis is dire:
Synchronous multi focal:
Childhood form almost always fatal.................within 6-8 months
Adult form almost always fatal .........................within 2 y
Asynchronous multifocal Not as uniformly fatal as synchronous type
5-Tumour response to chemotherapy
The only feature that CONSISTENTLY PREDICTS outcome is the degree of histologic necrosis
following induction chemotherapy:
> 90% chemotherapy induced tumor necrosis ...............90% disease free survival
< 90% chemotherapy induced tumor necrosis ...............15% disease free survival

53

6-Serum markers
A number of potential prognostic factors were identified and not validated yet these include:
1.Human epidermal growth factor.
2.Tumor cell ploidy.
3.Specific chromosome gains or losses.
4.Loss of heterozygosity of the RB gene.
5.Loss of heterozygosity of the p53 locus.
6.y expression of p-glycoprotein
y AlkPh. at diagnosis are more likely to have lung metastases
y LDH is bad prognostic value

Treatment of osteosarcoma
1. Operative: Which will be tailored according to the stage & extension of the tumour
2. Adjuvant therapy: Preoperative & Postoperative CHEMOTHERAPY
3. Adjuvant surgeries: As resection of a lung metastasis
1. Adjuvant therapy (Chemotherapy)

Chemotherapy administered preoperatively is referred to as induction (NEOADJUVANT)

Induce TUMOUR NECROSIS, particularly in the reactive zone (pseudocapsular region), where
microscopic satellite nodules reside
FACILITATING LIMB-SPARING resection with a wide margin.
INITIATES early treatment of microscopic and overt metastatic disease
Postoperatively Chemotherapy, when tumour burden is lowest, is called Adjuvant Chemotherapy
Typically, a combination of induction & adjuvant therapy is used in ttt of most HG sarcoma
The Huvos Grading system

Grade

Degree of necrosis

Prognosis

Grade I
Grade II
Grade III
Grade IV

<10% Necrosis
<90% Necrosis
>90% Necrosis
100% Necrosis

Bad
Bad
Good
Excellent

Chemotherapeutic agents used in the treatment of osteosarcoma

Agent

Mechanism of action

Side effects

Doxorubicin
(Adriamycin)

Doxorubicin intercalates at point of local

uncoiling of DNA double helix,
Also DNA & RNA synthesis

Cardimyopathy, trasnient ECG changes,

emesis, alopecia, mucositis,
BM supression

RF, PN, otoxicity, emsis, BM depression,

Cisplatin DNA synthesis via formation of
alopecia, z Mg
DNA cross-links; it binds to tumour DNA
Ifosfamide (Ifex) with
Ifosfamide causes cross link defect
Hemorrhagic cystitis, RF, emsis, BM
mesna (mesnex)
depression, alopecia, encephalopathy
DNA synthesis
Methotrexate
RF, mucositis, BM depression, CNS
Methotrexate is a folate antimetabolite; it
(Rheumatrex) +
purine synthesis & thymidine by binding
leucovorin ca
dihydrofolate
Mesna is a prophylactic agent used to prevent hemorrhagic cystitis; it has no intrinsic cytotoxicity. Mesna binds urotoxic
metabolite of ifosfamide inactivating it
Leucovorin is a tetrahydrofolic acid derivative that acts as a biochemical co-factor for carbon transfer reactions in the
synthesis of purines and pyrimidines. Leucovorin does not require the enzyme dihydrofolate reductase; therefore, it is able
to rescue normal cells and prevent severe myelosuppression and mucositis
Cisplatin (platinol)

54
2. Qperative Treatment:

Enneking stage I A & II A (Intra-compartmental lesions):

LIMB SPARING SURGERY

Enneking stage I B & II B (Extra-compartmental lesion):

LIMB SALVAGE VS AMPUTATION

Radical excision = Amputation OR Disarticulation

Enneking stage III (Lesion with metastasis): (Osteosarcoma with pulmonary metastasis)

1- RESECTABLE METASTASIS:

Resection of all macroscopic tumour tissue by a median sternotomy

Amputation or resection of the tumour
Intensive adjuvant chemotherapy

2- INOPERABLE METASTASIS
Chemotherapy
Palliative procedures

LIMB SALVAGE
Wide tumour Resection & Reconstruction of the skeletal gap by Common Modalities Of
Reconstructive Options
Surgical guidelines for limb sparing surgery:
1. BONE should be resected 3-4 cm beyond abnormal uptake as determined by Tc. MRI is useful
2. En bloc resection of normal MUSCLE cuff in all directions
3. Enbloc resection of all previous BIOPSY SITES & potentially contaminated tissues
4. Adjacent JOINT & capsule should be resected
5. There must be enough MUSCLE REMAINING for a functional limb to be reconstructed
6. There must be FREE NEUROVASCULAR bundle of tumour
7. There must be adequate soft tissue COVERAGE
Types:

Limb Salvage
Biological
Reconstruction
Distraction
osteogenesis

BG
Autograft

Allograft

Structural

Osteochondral

Metal
Reconstruction

Intercalary

Reimplantation

Heat

Non
structural

Resection
arthrodesis

APC

Freeze

Composite
Reconstruction
Auto Prosthetic
Composite
Irradiation

Allo Prosthetic
Composite

55

Types:
1. Biological reconstruction
A- Bone grafing
i. Autograft
ii. Allograft
1. Nonstructural allograft
2. Structural allograft
a. Osteochondral allograft
i. Hemicondylar
ii. Total candylar
b. Intercalary allograft.
3. Resection arthrodesis
4. Allograft prosthesis composite (APC)
iii. BG substitutes
B- Distraction osteogenesis:
i. Type 1: diaphyseal
ii. Type 2. Methaphyseal reconstruction
iii. Type 3. Epiphyseal reconstruction
iv. Type 4. Subarticular reconstruction
v. Type 5. Arthrodesis
C- Reimplantation: of the resected bone after devitalization of the tumour cells by:
i. Heat:
1. Autoclaving
2. Pasteurization
3. Microwave induced hyperthermia
ii. Freezing (Liquid nitrogen)
iii. Irradiation
2. Metallurgic reconstruction
3. Composite reconstruction:
A- Auto-prosthetic composite:

i. Extracorporeally irradiated autograft-prosthetic composite arthroplasty

ii. Autoclaved autograft-prosthetic composite arthroplasty
iii. Pasteurized autograft-prosthetic composite arthroplasty
B- Allo-prosthetic composite.

Relative contraindications to limb sparing surgery:

1. Major neurovascular Or Muscle involvement
2. Pathological fractures
3. Inappropriate biopsy sites
4. Infection
5. Skeletally immature patients LLD

LIMB SALVAGE IN SKELETALLY IMMATURE PATIENTS

The options for limb salvage in immature patient include:
1- Rotationplasty
2- Expandable endoprosthesis
3- Distraction osteogenesis

56
3. Radiotherapy:

Relatively radio-resistant tumour; but can be used for:

Palliation of local pain
Treat surgically inaccessible lesions
Treat painful metastatic deposits
May also be used pre-operatively to z the size and vascularity of the tumour
Prophylactic irradiation of the chest has not been shown to be effective

57

Osteoid Osteoma
Introduction

Benign skeletal neoplasm of unknown etiology is Composed Of OSTEOID AND WOVEN bone
The lesion initially appears as a small sclerotic island in a circular lucent defect. (N
NIDUS)
OO may REGRESS spontaneously. The mechanism of involution is not known ( infarction)

Aetiology

It is a REACTIVE BONE lesion, of unknown cause (not a neoplasm)

Theories of etiology of osteoid osteoma

1. Congenital.
2. Neoplastic: Jaffe Very slowly growing tumor
3. Rheumatic.
4. Reactive bone lesion (Now) localized reaction to infection
5. Inflammatory (Lymphocytes) sclerosing osteomyelitis :

Classification

Its SELF LIMITING nature

Its SHELL OF BONY SCLEROSIS
Its characteristic SITE
In some cases STAPHYLOCOCCI has been cultured

1. Cortical
2. Cancellous

3. Subperiosteal
4. Intra-articular

Cortical Osteoid Osteoma

Most common.
The radiolucent nidus is within the cortical bone
Fusiform cortical thickening + laminated periosteal NBF
Cancellous osteoid osteoma
Intramedullary location.
LITTLE SCLEROSIS occurs around the nidus
Sites:
Juxta-articular region of the Femoral Neck
Posterior elements of the Spine
Small bones of the Hands And Feet
Intra-articular osteoid osteomas
Intra-articular osteoid osteomas are DIFFICULT to identify
DELAY of 4 mo- 5 years before diagnosis is not unusual.
JOINT-SPACE WIDENING due to joint effusion or synovitis
DD:
inflammatory or infective arthritis
Nonspecific synovitis
Legg-Calve-Perthes disease

Subperiosteal osteoid osteoma

RARE form of the disease
ROUNDED SOFT-TISSUE MASS adjacent to a bony cortex & excavates
LITTLE SCLEROSIS occurs around the nidus

Sites:
Medial Femoral Neck (Juxta- or intra-articular)
Hands and Feet, in particular, the neck of the talus.

58
Incidence

12 % of benign tumors & 3% of all tumors.

:= 2:1
10-30 y.................................................. 75% (range from 5-55 y)
It is uncommon ................................... <5y or > 50 y
Pathology

Site:
METAPHYSIS or DIAPHYSIS .........75%

Appendicular skeleton...........65% (FEMUR & Tibia & Humerus; but occur in any bone)
Spine: ....................................12% (Posterior arch)
Hands: ...................................8%
Feet:.......................................4%
The skull and facial bones are involved exceptionally
80% of cases involve the cortical bone; 20% are intramedullary
Gross Pathology
Aggregate of RED, brown, purple or grey tissue (according to vv), GRITTY consistency
Encased in a dense cortical SCLEROSIS
Size of surrounding sclerosis may exceed the size of the principle lesion many times
Nidus does not exceed 2 cm (0.5-2 cm)
Described as a Cherry Piece inside the bone.
Microscopic
Nidus of OSTEOID TISSUE + WOVEN BONE TRABECULAE
HIGHLY VASCULAR CT matrix large dilated vascular channels.
GIANT CELLS and OSTEOCLASTS are frequently observed.
Jaffe described it microscopically to be formed of 3 characteristic regions:
Inner region..............vascular GRANULATION + OSTEOBLASTS.
Intermediate ............. CALCIFICATION & OSTEOID formation
Outer region .............Bone TRABECULAE in various stages of ossification
Clinical picture

The most important clinical characteristic of osteoid osteoma is PAIN

The site of involvement may be TENDER to touch or pressure.

Pain of osteoid osteoma............................ 95%

Characters:
y activity & at night with congesion & z by salicylates.
Not relieved by rest.
At 1st it is vague & later y in severity & becomes aching in character
Some times, severe enough to waken the patient.
In a few instances pain becomes severe & persistent (6y) threats of suicide
Site:
Usually the pain is at the site of the lesion
Femoral upper end; esp. the neck, may be referred into the leg as a LDP.
Cause:
Result from the lesion being very vascular numerous nerve fibers.
dema & increased tension result in of these nerve endings

59
Osteoid Osteoma Of The Spine
PAINFUL SCOLIOSIS ms spasms abnormal alignment (also kyphosis, torticollis & lordosis)
A may be CONCAVE TOWARD the Lesion.
May be ACUTE and is frequently initiated by PHYSICAL EXERTION.

Osteoid osteoma has been called the most common cause of painful scoliosis.
Osteoid osteoma affecting the hip

May cause pain simulating as that pain caused by LDP

Intracapsular lesion often considerable intra-articular inflammation look like arthritis
Marked weakness & muscular atrophy long-standing tumor may occur
X rays:
A circular or OVOID LUCENT defect is seen in 75% of patients.
Size is usually smaller than 1.5 CM in diameter
Variable degree of cortical & endosteal SCLEROSIS; according to the site:
Cortical and subperiosteal considerable sclerosis.
In medullary tumors
sclerosis is minimal or absent.
Subarticular & intracap
sclerosis is minimal & distant to the lesion.
Long-standing cases
more sclerosis.
Children also
more sclerotic response than adults
Intra-articular tumors .........................EFFUSION premature loss of cartilage.
intra-articular osteoid osteoma ...........OA (50% of intraarticular OO)
Spinal osteoid osteoma .......................SCOLIOSIS, kyphosis, or hyperlordosis.
children with a long-standing tumor .......OVERGROWTH of the involved bone
Regional OSTEOPOROSIS disuse.
CT:

MRI

CT is BEST diagnostic tool for nidus localization

The nidus signal is ISOECHOIC to muscle on T1 & HIGH on T2

BM edema around the nidus...............60%
Soft tissue edema ................................50%
Bone scan:
Tc99m shows INTENSE ACTIVITY at the site of the tumor.
Occasionally, DOUBLE-DENSITY sign (Nidus of y uptake on top of a larger area of radioactivity)
Differential diagnosis
1. Osteoblastoma
2. Sclerosing osteomyelitis
3. Sclerotic metastases
4. Osteosarcoma
5. Ewing tumor
6. Subperiosteal ABC
7. Stress fracture
8. Bone Infarct
Prognosis:
Good & if the patient withstand the pain for 5 y, it will disappear (self limiting disease)
Malignancy has not been reported after surgical treatment.

60

Treatment:
COMPLETE EXCISION the WHOLE NIDUS will provide cure

Partial removal of only symptomatic improvement but not cure & will recur
To avoid incomplete removal:
PRE-operative Good x-ray
INTRA-operative x-ray for the removed specimen (more clear than the whole bone)
Remove nidus ENBLOC + Prophylactic ORIF + BG (esp. in neck femur & long bones)
Techniques of osteoid osteoma ablation:
Percutaneously ablated by using radiofrequency (RF)
Ethanol
Laser
Thermo coagulation therapy under CT guidance
If sclerotic bone is removed and not the nidus pain will continue
Metaphyseal lesions its rapid healing; prophylactic ORIF is usually not necessary
Spinal lesions sometimes complete ablation & resection are not feasible

Intraoperative procedure for localizing the lesion.

1. X-rays
2. Multiplanar fluoroscopy
3. The use of a radioisotope probe
4. Intraoperative tetracycline fluorescence
5. The specimen should be radiographed in two planes intraoperative
6. Pathologist should examine the specimen carefully 5mm cuts to detect the nidus

Ivory Osteoma
= Compact osteoma = Ivory exostosis
Rare benign tumor arise as a localized thickening on the outer or inner surface of bone
Sites: usually in skull and medial tibia
Clinically: painless, hard lump, on the outer surface of the skull
If occur on the inner surface epilepsy
PXR: sessile dense bone well circumscribed edge
TTT: only when symptomatic

61

Osteoblastoma
Introduction

Osteoblastoma (OB) is an UNCOMMON primary neoplasm of the bone. It has clinical and
histologic manifestations similar to osteoid osteoma (producing osteoid, primitive woven
bone & fibro-vascular CT); therefore, some consider the two lesions to be variants of the
same disease, with osteoblastoma representing a giant osteoid osteoma. However, an
aggressive type of osteoblastoma has been recognized, making the relationship less clear
tiology
UNKNOWN.

Tumour is usually considered BENIGN

AGGRESSIVE VARIANT has been described in literature, histologic features as osteosarcomas

Incidence

4% of benign bone tumors.

1% of all primary bone tumors
Pathology

Site
40% in SPINE (17% of spine OB are in the SACRUM)
Posterior element
DIAPHYSES is another common site (LL more affected)
Sometimes Metaphyses are affected
Rarely epiphyses are affected
Pelvis, hands, feet, skull, clavicle, scapula, ribs, and
talus are also documented
Macroscopically
IRREGULAR OSTEOID mass
MORE VASCULAR, MORE GRITTY, but LESS SCLEROTIC than osteoid osteoma
Microscopic
Most important: IMMATURE BONY TRABECULAE & primitive WOVEN bone ossification
Lined OSTEOBLASTS
HIGHLY VASCULARIZED CT: The stroma has dilated capillaries & blood sinusoids
NIL MITOTIC ACTIVITY

It may simulates osteoid osteoma & osteosarcoma but:

1. larger than 2cm; but does not permeate the bone
2. y of osteoblasts hence its name.
3. Very low mitosis and cytologic atypia
4. Intercellular matrix varies in amount & maturation (from osteoid up to lamellae peripherally)
5. Rarely has a cartilaginous matrix ( osteosarcoma)
Clinical picture

Usually present with PAIN of several months duration:

Less intense
not worse at night
not relieved readily aspirin
If the lesion is superficial localized SWELLING and TENDERNESS.
Spinal lesions can cause PAINFUL SCOLIOSIS (this feature is more osteoid osteoma)
NEUROLOGIC deficits

62
X-RAY

Spine Osteoblastomas:
In the POSTERIOR ELEMENTS
WELL-DEFINED expansile lesion
50% contain MATRIX MINERALIZATION.
Long bones lesions:
66% in the cortex & 33% in medullary canal.
> 2 cm unlike osteoid osteoma
Well-circumscribed radiolucent lesion
Thin shell of peripheral NBF & reactive zone of bone
Computed tomography (CT):

Tumor delineation when the lesion is within the cortex

Extent of the lesion
Size of the lesion
Identify spine lesions clearly smaller and more accurate surgical resections.
Magnetic resonance imaging (MRI)

Provides information about the extent (NB. MRI findings alone are not diagnostic)
Detects soft tissue changes
Detects BM extension
Bone scintigraphy

y Tc99m is sensitive but not specific.

Many bone tumors, for example osteoid osteoma show the same type of focal activity

Differential diagnosis

ABC
Chondromyxoid Fibroma
Enchondroma and Enchondromatosis
EG
Pain
Size
Grossly
Mic

GCT
Osteoid Osteoma
Osteomyelitis
Osteosarcoma

Osteoid Osteoma

Osteoblastoma

More
< 2 cm whatever the duration
Only sclerosis of neighboring bone
Less osteoblasts

Less
Increase with long standing up to 7 cm
Some destruction & expansion of neighbor bone
More osteoblasts

Treatment

The treatment goal is complete surgical excision of the lesion.

The type of excision depends on the location of the tumor.
For non aggressive lesions:
Extensive intralesional excision, using a high-speed burr (remove tr + normal margin)
For aggressive lesions:
Wide resection is needed to remove all tumour-bearing tissue
This type of complete excision is usually curative for osteoblastoma.
Prognosis

Recurrence ~ 10-20% inadequate resection especially in spine lesions

63

V{wzx|v g
OSTEOCHONDROMA
o MULTIPLE HEREDITARY EXOSTOSIS
o ENCHONDROMA
o CHONDROBLASTOMA
o CHONDROMYXOID FIBROMA
o CHONDROSARCOMA
o

65

Osteochondroma
Osteochondroma is the MOST COMMON BENIGN BONE TUMOR. Mostly
asymptomatic, but they can cause mechanical symptoms
CARTILAGE CAP histology has the same defined zones in physis, namely
zone of proliferation, hypertrophy, calcification, and ossification
Incidence:
35% of benign bone tumors
10% of all bone tumors
< 20 years

:=3:1.
Etiology:
UNKNOWN; although, it was thought to be a PHYSEAL HERNIATION

(Idiopathic, traumatic or perichondrial ring deficiency)

It is a REACTIVE METAPLASTIC CARTILAGE that responds to the factors that
stimulate the growth plate and thus results in exostosis growth.
Recently, genetic karyotyping suggested that it is a TRUE NEOPLASTIC
process, not a reactive one. These findings and conclusions are in the
early stages of research
Pathology:

Site
Any bone that undergoes endochondral bone formation.
FEMUR .....................................30% / distal: proximal=3:1
Tibial ....................................20%
Proximal Humerus ...............20%
Hands and feet (10% of cases), scapula (4%), pelvis (5%)
Location:
Osteochondromas develop METAPHYSEAL ADJACENT TO PHYSIS
Then, grow AWAY FROM PHYSIS (as if isolated physis):
Rate of epiphyseal cartilage growth > osteochondroma
The muscle pull (even if not attached to it)
Growth of the osteochondroma STOP AT SKELETAL MATURITY
Macroscopic
May have a stalk PEDUNCULATED, or a broad base SESSILE
The stalk is made up of mature bone.
By definition, medullary canals of the bone and the stalk are
CONNECTED
CARTILAGE CAP - is thick in children- is replaced by endochondral bone at maturity

Microscopic:
Lesion is topped with a cartilage cap VARIABLE CELLULARITY.
The cap has an overlying fibrous layer MESENCHYMAL CELLS responsible for its growth
Cap cells are VERTICALLY oriented as in physis
Staging: Musculo-Skeletal Tumor Society (MSTS) staging for all benign lesions:
Stage I .................................. Inactive or static lesions (Latent)
Stage II ................................. Actively growing lesions (Active)
Stage III ............................... Actively growing & locally destructive (aggressive)

66
Clinical picture:

most commonly diagnosed INCIDENTALLY when PXR obtained for some other reason.
The second most common presentation is a MASS ( pain)
CAUSES OF PAIN IN OSTEOCHONDROMA:
1. Pressure on the overlying soft tissue.
2. Bursitis over the exostosis.
3. Irritation of surrounding tendons, muscles, or nerves can result in pain.
4. Stalk fracture
5. Cap infarction
6. Malignant transformation.
Complications:

1.
2.
3.
4.

Fracture
Neurologic Sequelae
Bursa Formation
Malignant Transformation

Fracture
Site: typically involves the stalk base esp. around the knee
Subsequently, callus formation causing band like sclerosis on PXR occurs healing.
No significant incidence of nonunion has been reported.
Interestingly, REGRESSION of solitary osteochondroma occur spontaneously and following #
Neurologic Sequelae
Peripheral lesions may compress nerves .... nerve palsy
PERONEAL affection foot-drop & RADIAL nerve Palsies are reported
Also central neurological manifestations are reported also central lesions
Bursa Formation
The commonest locations: (sites motion & friction):
SCAPULA ............................................ 50% of shoulder cases
LESSER TROCHANTER

Lined by synovium .................................... may become inflamed, infected, or hemorrhagic.

May contain chondral or fibrin bodies ....... 2RY SYNOVIAL CHONDROMATOSIS.
Malignant Transformation
Malignant transformation .......................... 1% of solitary lesions.
Plain X rays
METAPHYSEAL cartilage capped protuberance variable cap CALCIFICATION
There is CONTINUITY between the mass and the bone:
Broad ................................................ SESSILE osteochondroma
Narrow with a bulbous tip................ PEDUNCULATED osteochondroma
Characteristically there is CONTINUITY bet.the cortex and medulla of the parent & son

However, this contiuity is not apparent in:

Osteochondromas of flat bones with complex anatomy (i.e., pelvis, spine, scapula)
Sessile lesions
Pedunculated lesions usually POINT AWAY from the nearest joint
CT scanning:

1n certain bones, such as the pelvis and scapula useful to localize the lesion

67
MRI

Is only needed in cases in which malignancy is suspected

MRI is the modality of choice to assess the cartilage cap thickness.
Thick cartilage caps (>4 cm) + pain Suggestive

of malignant degeneration
Treatment:

INIDICATIONS FOR EXCISION

1. Painful bursae
2. Location recurrent injury
3. Significant deformity
4. Suspicion of malignant change
Guide lines:
Ideally, line of resection should be through ! base of ! stalk
Ensure that none of the cartilage cap or perichondrium is left otherwise recurrence
Ensure removal fibrous cover
Investigate very large lesions to exclude malign (MRI of choice in cartilage thickness)
Avoid damage to the physis
Avoid deep dissection violate normal tissue
Avoid superficial dissection leave a pathology
The resected surface of the host bone can be rasped smooth
Bone wax can be packed on the cut surface to stop bleeding
Prognosis:

For solitary osteochondromas excellent (local recurrence rate < 2%)

Poorer outcomes are associated with:
Surgical exposure
Secondary bone deformity due to lesion location (in the multiple hereditary)
Recurrence:

The local recurrence ~ 1.8%

Causes of recurrence after excision:
1. 1f you left the cartilage cap
2. If malignant

68

Multiple Hereditary Exostoses

Introduction

Diaphyseal Aclasis

Multiple Hereditary Exostoses is an inherited disorder of bone growth characterized by

growth of multiple exostoses (benign cartilage-capped tumors) that grow out of metaphyses.
Inheritance & Incidence

AD
65-75% have MUTATIONS at EXT1 gene & ~ 21-30% at EXT2 gene
Peak at 3y (nearly all patients are diagnosed by 12y)
HME Findings y with:
AGE: at birth5% & 12 Y96% & adult75% have an evident deformity
MALES > females.
HME Malignant risk y age
Pathology

Sites: Any bone can be affected

KNEE ..................................................................(70%)
Humerus ...........................................................(50%)
Forearm ............................................................(50%)
Ankle & scapula ...............................................(50%)
Pelvis, shoulder, ribs
Face and skull are generally unaffected
Location
Multiple & Usually bilateral & symmetrical
JUXTA PHYSEAL METAPHYSES (distance to epiphysis y growth)
From the SURFACE OF FLAT bones
Gross:
EXOSTOSES bony growths & by definition they must have:
Cartilage-cap small punctate calcifications
Their own cortex, medulla, and physis but continue those of mother bone
Grow away from physis OSSIFY and STOP GROWING by maturity
An exostosis may be SESSILE or PEDUNCULATED
ABNORMAL BONE REMODELING short, bowed bone wide metaphysis
Clinical picture:

The affected individual has 6 EXOSTOSES; typically BILATERAL, SYMMETRICAL.

DEFORMITY:
ABNORMAL REMODELING shortening, bowing, widening of metaphyses
Radius &Tibia
BOWING of the forearm or leg, is the commonest y Ulma
& Fibula deformities

Hand deformity shortened metacarpals is common

LLD marked interference with growth in length of the affected bones
MASS may cause:
Compress or stretch peripheral nerves..............pain, sensory, motor
Displace nerves and vessels ..............................complicating attempts at surgery
Irritate overlying ms & tendons ........................pain and loss of motion
Large exostosis impinging a joint .....................mechanical blocks to motion
Large pelvic exostosis.......................................urinary or intestinal obstruction
COMPLICATIONS: e.g. rapid y in size and pain malignant change

69

MHE has 3 main characteristics

1. Exostoses
2. Barrel shaped metaphysis failure of remodeling (Tubulation, funnelization or cylinderization)
3. Short stature (or even Dwarfism)

PXR

Multiple EXOSTOSES (description as those of solitary ones)

~ 70% have a clinically apparent exostosis about the knee, suggesting that PXR knee to
detect non-palpable exostoses may be a sensitive screening test to detect mildly affected
individuals.
Complications
1. Deformity
2. Fracture
3. Neurologic Sequelae
4. Bursa Formation
5. Malignant Transformation
6. Vascular

Cosmetic and Osseous Deformity

Wrist: ..................................................Ulnar tilt of radial articular surface, Ulnar deviation
Forearm: .............................................Ulnar shortening, radial bowing
Elbow: ................................................radial head dislocation
Hip: ....................................................coxa valgus
Knee: ..................................................genu valgus
Ankle: ................................................tibiotalar tilt (valgus deformity)
Significant LLD & short stature
Other skeletal abnormalities may occur:
Shortening of 4th and 5th metacarpals
Supernumerary fingers and/or toes
Madelung / reversed Madelung deformity
Vascular Compromise
Vessel displacement is common large lesions but asymptomatic
Stenosis
Occlusion & thrombosis: either arterial or venous. Most frequently seen in POPLITEAL vv
Pseudo aneurysm formation.

Clinically:
Pain, swelling
Rarely claudication
Palpable pulsatile mass usually affecting a young patient.

70

Malignant Transformation (most serious)

Usually occurs in adulthood
The loci on chromosome 8 & 11 loss of heterozygosity associated malignant change
Symptoms suggesting of malignant change:
Rapid y in SIZE
Rapid y in PAIN
Any pain and growth AFTER skeletal maturity
Sign of malignant change:
Suspected more in PELVIS and shoulder
BULKY CARTILAGE CAP (best visualized MRI or CT) > 3cm
y Tc99m UPTAKE after skeletal maturity
Types:
CHONDROSARCOMA arise from the cartilage cap
Rarely osteosarcoma arise from the base of the stalk
Usually solitary and LG ......................85% (multifocal & dedifferentiated may occur)
Incidence
8% of all chondrosarcomas
Change rate is .....................................0.5-20%
Sites more prone: ................................pelvis, hips, and shoulders lesions are
Age: (unusual before 20y)
HME ......................................25y
Solitary osteochondroma .......50y (usually)
Prognosis

Exostosis begins in childhood

Stops growing when nearest epiphyseal center fuses

71

Enchondroma
Benign tumour of cartilage originating within the medullary cavity
Periosteal form originates in the periosteum and erodes into the cortex
Incidence

10% of benign bone tumours

Peak incidence ................................ 10-50Y
Long bone chondromas ...................> 30 years
May be SOLITARY or MULTIPLE (Olliers, Mafuccis)

Pathology

Site:
o
o
o
o

HANDS AND FEET ....................>50% occur in

Femur & humerus .................15% each
METAPHYSEAL

Solitary swelling ...................75%

Macroscopically - BLUISH white well DEMARCATED, ENCAPSULATED
and often LOBULATED gritty tissue
Microscopically - HYPOCELLULAR; NESTS of mature cartilage cells,
nuclei are small and uniform, no atypia calcification
Need to section all areas of the specimen as sarcomatous change
may occur in a benign lesion
PERIOSTEAL FORM less common
o Similar pathology but more cellular
o Proximal humerus near deltoid insertion
o Never turn malignant
OLLIER'S more cellular ......................50% malignant transform
MAFFUCCI'S haemangiomata ...........100% malignant change

Clinically

Present as FRACTURES .......................60%

LUMP, or as INCIDENTAL finding
Cortex remains intact unless fracture

Radiology

SCALLOPED EROSION of endosteal surface

flecks of calcification ...................... 'GROUND GLASS'
Periosteal form (juxtacortical) ......... SHALLOW CRATER

lined rim of reactive bone, elevate periosteum;

the lesion it self may not show on the PXR
Oliers & Maffucci........................... SLED RUNNER TRACKS

Treatment

Observe x-ray 6mo & 1y after presentation

Latent .............................................. CURETTAGE + BG
Active...............................................Recurrence may be better than morbidity of en block
Periosteal form................................. MARGINAL EN BLOC excision

Prognosis

Risk of malignant change in Olliers is 50% & in Maffucci is 100%

Recurrence is higher active lesions (GII) and periosteal form

72

Chondroblastoma
Incidence

1% of benign bone tumours

: 2:1
Peak age 10 - 20 Y ................................. (rare over 30 years)

the adult counterpart of chondroblastoma is giant cell tumour

Pathology

Arises from CHONDROBLASTS

Usually ACTIVE BENIGN lesion (Stage 2)
Sites:
o UPPER HUMERUS ......................15%
o UPPER TIBIA .............................15%
o Upper femur..........................15%
o Lower femur .........................15%
o EPIPHYSEAL but may expand into metaphysis
Mac: PINKISH grey LOBULATED tissue, may be haemorrhagic
Mic: Richly cellular MNGC + CHONDROBLASTS (polyclonal or round)

Clinically

Present with PAIN of increasing severity

X-Rays

OPEN PHYSIS
ECCENTRIC, EPIPHYSEAL, WELL DEFINED, LYTIC area placed in the or across the physis

No reaction in surrounding bone

50% ..................................................central CALCIFICATION
50% .................................................. LINEAR PERIOSTEAL REACTION
Bone scan increased uptake at margins

Differential Diagnosis:

GCT (adults)
ABC (histology similar)
clear cell chondrosarcoma
epiphyseal osteomyelitis

Treatment

Curettage & bone grafting (15% recurrence)

AVOID JOINT PENETRATION because chondroblastoma cells will grow in joint fluid
Use cryotherapy if extension intra capsular to avoid excision of joint

Prognosis

Probably no chance of malignant change

Recurrence 15%

73

Chondromyxoid Fibroma

Benign tumor of bone characterized by formation of variable amounts of chondroid,

fibromatoid & myxoid elements

Incidence

2% of benign bone tumours

Peak age 10-30Y ...............................(75%)

Pathology

May develop from a REMNANT OF THE GROWTH PLATE?

Sites:
o lower extremity.....................75%
o TIBIA .......................................50%
o Forearm.................................10%
o Usually ECCENTRIC METAPHYSEAL lesions
Macroscopic:
o firm LOBULATED JELLY like areas of MUCOID masses
o areas of CHONDROID and MYXOMATOUS tissue
Microscopic:
o
o
o
o

MNGC
MACROPHAGES
MONOCYTES with condensations of cells on the periphery
Usually NO BONE OSTEOID

Clinically

Present with a CHRONIC PAIN

X-Rays

Rounded or oval LYTIC area

Usually ECCENTRIC METAPHYSEAL ; but may cross the growth plate
SHARP outline
SCLEROTIC rim
SCALLOPED margin and thin cortex

Treatment

EXTRA CAPSULAR MARGINAL EXCISION...almost no recurrence

If skeletally immature ...................... WAIT until maturity

Prognosis

Malignant change is reported...........should be excised

74

Chondrosarcoma

Primary malignant tumour whose CELLS PRODUCE CARTILAGE MATRIX

May arise DE NOVO (usually IM) or 2RY to an existing benign tumour

Incidence

th

10% of primary bone sarcoma (4 common sarcoma)

AGE: ................................................. 30 - 60 years
Male : Female ................................. 2:1

Pathology

Sites:
1. PELVIS ....................................30%
2. Prox Femur ..........................20%
3. Prox Humerus ......................10%
4. Ribs ......................................10%
5. Rare in hand but it is the commonest sarcoma there
6. Often metaphyseal
Conventional Macroscopic appearance:
o EXPANDING lesion cortical DESTRUCTION, soft tissue extension
o Grayish HYALINE FUSED NODULES areas of CALCIFICATION
Conventional Microscopic appearance:
o Cellular PLEOMORPHISM + focally CALCIFIED matrix
Histological Classification:

Grade I

(30-35%)

Grade II
(40-50%)

Clinical Behavior
SLOW GROWING
LOCALLY AGGRESSIVE
Metastasis in 20%

Grade III METASTASIZING

(15-30%)

Metastasis in 70%

Chondrocytes

Matrix

Small, dark nuclei, scant cytoplasm, in clones

Occupy lacunae with odd number of cells
No mitoses
Larger and paler nuclei, y cytoplasm
Mild pleomorphism
Very rare mitoses
Large and vesicular nuclei, yy cytoplasm
Marked Pleomorphism
Mitoses: 2 /10 HPF

Low cellularity
Abundant matrix
Calcification
More cellular
Less Matrix
Focal myxoid change
Intense hypercellularity
Sparse matrix

Topographic Classification:
1. INTRAMEDULLARY either DENOVO or 2RY to enchondroma:
Olier's & Maffucci's are at much higher risk
2. SURFACE usually 2RY to osteochondroma:
y SIZE, FUZZY, CAP > 4cm, BASE > 6cm diameter

Pathological Classification:
1. CONVENTIONAL

2. LOW GRADE:

1. CLEAR CELL (MALIGNANT CHONDROBLASTOMA)

2. JUXTA-CORTICAL

3. HIGH GRADE:

1. MESENCHYMAL CS
2. DEDIFFERENTIATED CS

Metastasize to lung

75
Clinically

Presents with CONSTANT PAIN or increased size of a pre-existing lump

Most common malignant tumour of the hands and face in middle aged patients
Metastatic deposits are infrequent and usually go to lung

X-Rays

Variable appearance with 60 - 70% have calcification and 50% have sub periosteal NBF
Large CYSTIC lesion cortical DESTRUCTION + SCALLOPING & EXPANSION
CENTRAL CALCIFICATION

Treatment

These tumours tend to METASTASIZE LATE therefore attempt wide local excision initially
Radiotherapy useful for the treatment of surgically inaccessible sites however are relatively
CHEMO & RADIO-RESISTANT

Treatment protocol as those of malignant osteosarcoma = LIMB SALVAGE

Prognosis

Dependant on grade:
Low grade ........................................80% 5YSR
High grade........................................20% 5YSR

DEDIFFERENTIATED CHONDROSARCOMA
HG chondrosarcoma + areas of osteosarcoma, fibrosarcoma, or MFH (in this frequency)

PXR: aggressive lucent area on previous classic chondrosarcoma

CLEAR CELL CHONDROSARCOMA
LG variant of chondroblastoma

Affects epiphysis of long bones, usually UPPER FEMUR

Pathology
Numerous cells abundant CLEAR VACUOLATED CYTOPLASM
Scattered MNGC
Scant CHONDROID matrix
May be confused with GCT, Chondroblastoma, or renal clear cell tumour
PXR:
Well CIRCUMSCRIBED LUCENT defects often with thin SCLEROTIC border
MESENCHYMAL CHONDROSARCOMA

Rare HG
Usually occurs in the RIBS or JAW
Age usually 10 - 30 years
Sheets of small undifferentiated cells resemble HG EWINGS + focal LG CHONDROID matrix
Metastasis usually to lung

77

V|v _x|
(1) Simple Bone Cyst
(2) Aneurysmal Bone Cyst
(3) Giant Cell Tumor

79

Unicameral Bone Cyst

(Simple Bone Cyst)
Definition:

The simple bone cyst is a common benign lesion, is

characterized by fluid-containing cyst at the metaphysis of long

bones, and usually of unknown cause
The unicameral bone cyst is probably not a true neoplasm.
Cysts that abut a physis have in the past been termed active & have
tended to recur with greater frequency than those separated from
the plate by normal bone i.e.: inactive.
Epidemiology
AGE: ...................................................... 5- 15 years.
After 10y ............................................... z recurrence rate
SEX: : ............................................... 2 : 1

The pathogenesis: (unknown)

V ENOUS O BSTRUCTION A ND B LOCKAGE
Venous obstruction and blockage of interstitial fluid drainage, in an area of rapidly

growing and remodeling cancellous bone lead to the development SBC

T RAUMATIC THEORY :
Because the lesion occurs in a growing bone, it is probably the result of mechanical trauma

to an area of bone growth in the physeal line creating a defect at that point failure of
endochondral bone formation resulting in a hiatus present as a unicameral bone cyst.
In favor of this theory ........................... age group affected.
Against this theory ................................ cysts that cross the mid-diaphyseal point
H EMORRHAGIC THEORY
Some suggest that solitary cyst is the result of a hmorrhage into the metaphysis

organizes by fibrous wall formation; while the blood volume clots then liquefies
The surrounding fibrous capsule acts as a semi-permeable membrane & if the osmotic
pressure of the intra-cystic content is higher than that of the surrounding tissue, water is
drawn into the cyst y intra-cystic pressure and the cyst expands pressure erosion on
the surrounding cancellous tissue & then cortex.
Extra & intra cystic densities ................ eventually stabilizes latent stage
Against this theory ................................ pts hemophilia do not show a y incidence of SBC
FAILURE OF BONE FORMATION :
If the cyst is a defect resulting from failure of bone formation by an aberrant part of the

physis defect involves the diaphyseal portion supposed to be formed by that part
The cartilage growth is no longer converted to bone; while the surrounding bone is

remodeling normally false appearance of expansion that never been wider than the
epiphyseal line & this is a diagnostic feature.
NBF laid down as the child matures & the cyst then occupies an area towards diaphysis
In adult state many cysts are obliterated radiographically by NBF on the overlying cortex.
It becomes smaller than the width of the adult bone

80
Pathology

SITE
Tubular bones ...................................... 90-95% of patients.
Location ................................................ PROXIMAL METAPHYSEAL
Diaphyseal in only ............................... 4-12% of patients
Commonest site..................................... PROXIMAL HUMERUS & UPPER FEMUR.
MACROSCOPIC:
There is always ..................................... ZONE OF NORMAL BONE between the cyst & physis

represents the amount of longitudinal bone growth since the establishment of the lesion.
It has been stated that cysts that appear very near the line are still actively growing & that
therapy at this time ............................... result in a RECURRENCE
As the bone continues to grow.............. CYST DRIFT to mid-diaphysis (never cross it)
The outer cortical shell ......................... covered by an intact periosteum
The cyst contains ................................. CLEAR SEROUS FLUID (Occasionally, blood if #)
The cyst is lined by .............................. PALE BROWN MEMBRANE of few mm thickness
After a fracture ..................................... Fibrous septa form MULTILOCULAR APPEARANCE.
MICROSCOPIC:
There are no pathognomonic features about the tissue that is curetted from the bony walls
Lining membrane is formed of ............ young fibrocytes + deeply stained fibrin + collagen
Hemosiderin pigments
Xanthoid cells
MnGC

Clinical picture:

Usually ASYMPTOMATIC SBC develops very slowly

Rarely causes mild PAIN
Pain occurs when the overlying cortex is thinned to the point of pathologic fracture.
INCIDENTALLY, a bone cyst is discovered in radiographic surveys done for another reason.
50% of patients have PATHOLOGICAL FRACTURE

X rays

WELL-DEFINED, GEOGRAPHIC, LYTIC lesion with narrow transition zones

A thin SCLEROTIC MARGIN is a typical finding.
INTRAMEDULLARY METAPHYSEAL immediately JUXTA-PHYSEAL (occasionally diaphyseal)

The distance to physis varies ................ depending on the duration

Although EXPANSION & THINNING ......... cortex usually not wider than metaphysis
The long axis of the lesion.................... parallels that of the bone
Pathologic fracture lead to the migration of a fragment of bone to a dependent portion of
the fluid-filled cyst ............................... pathognomonic FALLEN FRAGMENT SIGN
Some may have..................................... multilocular appearance

81
Differential Diagnosis:

Fibrous dysplasia.
Non-ossifying fibroma.
Giant cell tumour.
Enchondroma.
Osteitis fibrosa cystica.
Neurofibroma.

Treatment:
Surgery is indicated primarily:
o
o
o

Younger age group

Impending #
Coxa vara deformity or any deformity (z bone strength)

Modalities:
1. Traditionally.......................................... CURETTAGE + BG
Recurrence that require redo ........... 25%.
Recurrence z with ......................... high-speed burr on the cyst walls after curettage
Cauterization with phenol following curettage has not reduced the recurrence.
2. Currently successful healing is reported by METHYL-PREDNISOLONE ACETATE (200 mg)

injection into the cavity

The mechanism of action:
o Unclear, reparative response to the injection process
Advantages:
o Shorter operating time
o less bleeding
o Minimum hospital stay
o Minimum rehabilitation time
Disadvantages:
o unpredictable healing
o Incomplete healing even after multiple injections
o Failure rate in WB bones...... high
Radiographic evidence of a good response:
o z cavity size
SBC
o y radio-opacification of the cyst
-5-15y male
o Cortical thickening
-Upper Humerus & Femur
Juxtaphyseal Metaphyseal
o Osseous remodeling
Complications:
Pathological fracture:

Common............................... 50%
Callus of repair .................... exerts healing influence
Considered as ...................... diagnostic & curative
Malignant transformation ............... very rare
o
o
o

-Clear fluid filled cyst

-Fibrous Wall
-Asymptomatic
-50% pathological #
-PXR: Fallen Leaf
-Sclerotic edge
-Treat Curette BG or
steroid injection

82

Aneurysmal Bone Cyst

Introduction
WHO definition ABC is a benign expanding cystic osteolytic tumor lesion consisting of

blood-filled spaces separated by CT septa containing trabeculae or osteoid tissue and

osteoclast giant cells
Although benign, the ABC can be a rapidly growing and destructive
Incidence

True incidence is difficult to calculate spontaneous regression & silent cases

Considered ........................................... rare especially in very young age
SEX ........................................................ SLIGHT >
AGE: ....................................................... 2nd decade 15Y (70%); but it occurs at any age

Etiology:

Vascular malformation within the bone unclear cause

Three commonly proposed theories are as follows:
1. ABC may arise de novo = PRIMARY ABC associated gene 17p13
2. ABC may arise in an area of prior trauma
3. ABCs caused by a reaction 2ry to another bony lesion = SECONDARY ABC:

This theory was proposed y incidence of associated tumors in 30 %

Giant cell tumors are the most commonly present
Other benign and malignant tumors are found:
FD
Giant Cell Tumor
SBC
NOF & EG
Osteoblastoma
Telangiectatic Osteosarcoma
Chondroblastoma
Chondrosarcoma
Metastatic carcinoma
Chondromyxoid
Pathogenesis
The true pathogenesis is unknown
Different theories:

Vascular malformations
AV fistulas
Venous blockage
Altered hemodynamics
The vascular lesions y pressure / expansion / erosion / resorption of bone
Local hemorrhage formation of reactive osteolytic tissue
o
o
o
o

Pathology
SITE:
ABCs may affect any bone in the body
ABCs most common ............................ 80%LONG BONES spine flat bones (pelvis)
Mainly................................................... ECCENTRICALLY METAPHYSEAL
subperiosteal, diaphyseal, epiphyseal ... rare
2ry lesions tend to occur in the sites where 1ry lesions typically arises.

83
MACROSCOPIC APPEARANCE:
Cystic blood-filled cavity charcteristic BLOOD-SOAKED SPONGE appearance
marked expansion or ballooning of the bone
The cavity is divided into cavernous spaces by intertwining fibrous mesenchymal septa
Thin subperiosteal shell of NBF surrounds the lesion
Intra-op.: when blood is sucked refill quickly (WELLING UP) = (POURING OF BLOOD)
It has no connection systemic circulation; but its blood comes from septal capillaries
MICROSCOPIC APPEARANCE:
Large vascular spaces
Fibrous septa contain:

Macrophages containing hemosiderin

Fibroblasts
Osteoid areas
Uneven distribution of multinucleated giant cells
No muscular or elastic layer in the cavernous walls
Areas of NBF
Mitotic figures are common, but no atypia
Clinical picture:

Patients usually present with PAIN ....... 90%

A MASS may ....................................... Rapidly enlarged
NEUROLOGIC symptoms ......................... secondary to pressure on a nerve & spinal ABC
PATHOLOGIC # ........................................ 10-20% (y in spinal lesions)
GROWTH DISPROPORTION ......................... disruption of the physis

Plain X ray:

Eccentric ( central or subperiosteal), cystic lytic lesion

Expansion of bone ............................... BLOWN-OUT, BALLOONED
Compressed trabeculae ...........................SOAP-BUBBLE & PENCIL IN CUP
Periosteal NBF rim .................................EGG-SHELL appearance
Spine:
Loss of pedicle of involved vertebrae
Posterior elements is preferred location

CAPANNA RADIOLOGICAL CLASSIFICATION:

Type
Site
Type I
Type II
Type III
Type IV
Type V

Q:

Central metaphyseal
Entire segment of a bone
Eccentric metaphyseal
Subperiosteal diaphyseal
Metadiaphyseal

Cortical Expansion

Cortical Erosion

Min
Marked
Min
Min
Marked & push periosteum to soft tissue

+/+
+

Malignant ABC is a term used for multiple recurrent aggressive ABC

Rarely present malignant change or pathological #

CT scanning:
Same PXR finding
Internal SEPTATION (ie, calcified rim, eggshell appearance)
FLUID-FLUID LEVELS; these are most often found in the ABC, but they are not exclusive to it.

84
MRI:
As CT scan, but they can more specifically reveal blood & expansion into soft tissues
The appearance on imaging studies has been divided into 4 PHASES OF PROGRESSION:
Phase
Progression
Cortical Expansion Cortical Erosion
Trabeculation
I- Initial (incipient)
II- Growth

-Starts slow
-Rapid

III- Stable

-Plateau

IV- Healing

-Progressive calcifn

-Little & saucer shape

-Severe & blown out
-Minimal periosteal reac.
-Classic Soap-Bubble
-Egg-shell surround
-Diminished

-Little
-Massive destruction
& bone lysis
-Classic

-Min
-Destruction outpace
trabeculation & NBF
-Numerous

-Healing

-Coarse irregular

Radiographic differential diagnosis includes:

Giant cell tumour:
Age
Site
Microscopic
Macroscopic

ABC

Giant Cell tumour

Young
Metaphyseal Eccentric
Cavernous blood spaces
fibrous septa + Giant cells
Blood-soaked sponge
Ballooning

Adult
Epiphyseal central may extend to metaph.
Spindle cells
Giant cells + Bl. vessels
Liver piece
Lack of expansion

ABC

SBC

young
Eccentric Metaphyseal
Blood-soaked sponge
Ballooning
Egg shell

younger
Central Metaphyseal then diaphyseal growth
Clear yellow fluid
Cortical endosteal erosion & expansion
Cortical continuity

SBC
Age
Site
Macroscopic

Telangiectatic osteosarcoma

Difficult to distinguish radiographically from an aggressive ABC

FD
Osteoblastoma

May have a soap bubble expansile appearance

No fluid level on CT/MR
Treatment:

I. Stage 1 (latent ABC):

Can be treated with ............................... intralesional CURETTAGE +BG

II. Stage 2 (Active ABC):

Is treated by........................................... INTRALESIONAL EXCISION + BG

The difference between curettage and excision is that excision involves wide unroofing of

the lesion through a cortical window by carefully abrading all surfaces with a high-speed
burr and possibly local adjuvants such as phenol, PMMA, or liquid nitrogen
The adjuvant therapy extends the area of treatment beyond that which physically can be
excised. The use of liquid nitrogen, phenol, and polymethylmethacrylate may achieve an
extended area of treatment. The adjuvants involve the use of chemical, freezing, or thermal
means to cause bone necrosis and microvascular damage to the walls of the physically
excised cyst, disrupting the possible etiology.

85

III. Stage 3 (Progressive ABC): or in expendable bone

EN BLOC or WIDE EXCISION is typically reserved for those are not amenable to intralesional

excision (e.g. extensive bony destruction) and has a recurrence rate of about 7%.
RECONSTRUCTIVE options after wide excision include:
Structural allografting
Autografting
Reconstruction with either endoprosthesis or APC.
IV. ABC in huge lesions of spine and pelvis:
This is an exception for the rule of irradiation to malignant tumors only
Irradiation is reported to induce healing using megavoltage of 30Gr
Operative intervention could be very difficult & associated massive fatal bleeding
Liquid nitrogen
Is the most popular adjuvant. After the ABC is exposed and a window is opened, liquid
nitrogen is poured in the cyst through a funnel
The surgeon should be sure to leave the window open, allowing the gas to escape.
A total of 2-3 cycles of freezing & thawing should be used to obtain maximum necrosis
Surrounding tissue, especially the bundle should be protected
Phenol:
Less used because of its poor penetration of bone compared with that of liquid nitrogen
However, phenol has had some success & is easy to use
Phenol is simply applied by soaked swabs, and remaining phenol is sucked
Cavity is filled absolute alcohol isotonic sodium chloride solution.
Polymethylmethacrylate (PMMA):
Induce bone necrosis from its thermal properties has been questioned in the literature
However, PMMA has the benefit of rendering a large lesion mechanically sound
If used subchondral joint surface should be protected by cancellous grafts or Gelfoam
Indications of PMMA:
1. Huge cavity
2. Old age
3. Weight bearing bone
Selective arterial embolization:
By using angiography, an embolic agent is placed at a feeding artery to the ABC, cutting
off the nutrient supply and altering the hemodynamics of the ABC.
Various materials, such as springs and foam, have been used to create the emboli:
Selective arterial embolization is indicated in:
1. Difficult locations
2. May be performed within 48 hours prior to surgery to z hemorrhage
PROGNOSIS:
The prognosis for an ABC ................... generally EXCELLENT.
The most common problem ................. RECURRENCE
The overall cure rate ............................ 90-95%.

86
Recurrence
st

Usually happens within......................... 1 y after surgery

All episodes occur within .................... 2 y
Patients should be monitored till .......... 5y till maturity to

detect early recurrence & to make sure that the tumor does
not cause deformity or interfere with growth.
Recurrence y :
Capanna I , II ............................. 24%
Capanna III, IV, V ..................... <5%
younger age & open growth plate
Metaphyseal lesion
Stage 3 aggressive lesions

ABC

-15y female
-Unknown cause
-Eccentric Metaphyseal long bone
-Blood soaked sponge
-Welling of Blood
-CP: Pain, Pathological #, Mass
-PXR: Soap Bubble, Egg-shell,
Pencil in cup, Ballooning
-Capanna PXR classification
-MRI Fluid-fluid level, classification
-ttt: Excision (accordingly) + BG

Ganglion cyst of bone

Occur typically in middle-aged men at the ends of long bones, particularly the distal tibia.
Considered intra-osseous extensions of soft tissues ganglia
Subperiosteal ganglia have also been reported .
Treatment is by local excision of overlying soft tissues & curettage of the involved bone .
Recurrences is unusual

87

Giant Cell Tumor of Bone

= Myeloid sarcoma = tumor of myeloplaxus = osteo-blasto-clastoma = osteoclastoma
GCT of bone has been described as the most challenging benign bone tumor. Although
benign, GCT shows a tendency for significant destruction, recurrence, and metastasis
Cell of Origin
Supporting cells of bone CT & not osteoclasts (both originate from mesenchymal cells)

Theories of the etiology:

1.
2.
3.
4.

Traumatic theory
Neoplastic theory
Chronic inflammation
Localized form of parathyroid osteodystrophy

Incidence

5% of all tumors
20 % of all benign bone tumors
Race: Affects all races esp. .................. China & southern India
Sex:
> (Unlike the majority of osseous neoplasms)
In contrast, malignant GCT is more in men (3:1)
Age:
The vast majority ...................... SKELETALLY MATURES
80% ........................................... 3RD DECADE (20-50y)
< 14y ......................................... rare (1-3%)
> 50y .......................................... 10%
Pathologic Features

SITE: ABOUT THE KNEE (50-65%)

The single commonest site:

Distal femur .................. (30%)

Proximal tibia ............... (25%)
Distal radius ................. (10%), sacrum (5%), prox.humerus (5%)
GCT also occur in:
Sesamoid bones............. patella
Apophyses ......................eg, G.trochanter is considered epiphyseal equivalents

LOCATION
ECCENTRIC META-EPIPHYSEAL

85-99% of lesions reach .................1 CM SUB ARTICULAR (most important diagnostic feature)
Site of origin is controversial .........METAPHYSEAL SIDE OF THE EPIPHYSEAL PLATE.

MACROSCOPIC

Relatively large lesions extending to the articular surface.

Cortex is expanded / periosteum is not breached / soft-tissue extension is not uncommon
Grossly soft, friable, fleshy, chocolate brown, yellowish-orange hemosiderin
Areas of:
Fibrosis.......................... (white)
hge & blood................... (red to brown depending on chronicity)
Xanthomatous .............. (yellow).
Blood filled cystic cavities

88
MICROSCOPIC

Contains diffuse OSTEOCLASTIC GIANT CELLS, hence the old name osteoclastoma. Giant
cells may suggest the Dx, but not specific; as many lesions have similar appearance.
Dx need clinical & PXR & histologic evaluation of mononuclear component
GCT = large of GIANT CELLS IN A BACKGROUND OF MONONUCLEAR CELLS
MULTINUCLEATED GIANT CELLS (MnGC):
There are ...................... 30 Giant cell/HPF
Cell contains ................. 30 nuclei
Cell diameter ................ 30
Distributed throughout the lesion & their concentration
varies from tumor to tumor. Some tumors have many,
whereas others have a few MnGC nestled in swirls of
spindle-shaped stromal cells
MnGC Concentration.... not related to recurrence or metastasis
MnGC invade vessels .... 3% (although aggressive, not related to prognosis)
MONONUCLEAR cells:
Round, oval, or polygonal & may RESEMBLE HISTIOCYTES
Fusion of these mononuclears may result in giant cell formation
Mitotic figures may be abundant
STROMA:
VASCULAR numerous thin-walled capillaries, often small areas of hge.
Stromal cells NUCLEI ARE INDISTINGUISHABLE from MnGC (helpful in DDx)
May be associated 2RY ABC but solid areas of classic GCT
Small FOCI OF OSTEOID matrix (never chondroid) produced by the stromal cells
Extensive hemorrhage, pathologic fracture, or previous surgery can alter
significantly the usual histologic picture of GCT resemble a 1ry sarcoma
According To the Predominant Growth Character 5 Types Are Present:

1.
2.
3.
4.
5.

Maroon color type

Cystic type
Telangiectatic type
Fibrous or white (Myeloid)Type........... Lower radius
Yellow (Xanthometaous ) type

SPREAD

Metastasize to the lung............... 3%

The metastases appear................ clusters of GCT located within the lung
Metastasis appears ..................... 3-5 y after detection (detection may take 10y)
The natural history of lung metastases is unpredictable; may regress, remain stable,
continuously grow slowly, or rapidly progress have been reported
Clinically:

Nonspecific and include (in order of decreasing frequency) pain, swelling, z ROM
PAIN is usually of several months duration / z by rest.
Related to associated PATHOLOGIC # acute onset of pain (10%)
NEUROLOGIC symptoms may be associated with spine lesions

89
Differential Diagnosis
DDx of GCT is extensive, including (but not limited to):

Brown tumor
Osteoblastoma
Chondroblastoma

ABC
NOF
Osteosarcoma giant cells.

These lesions can be difficult to distinguish from one another, particularly FNAC or with frozen

section specimens careful clinical, pathologic, and PXR correlation. This is particularly true of
brown tumor of hyper PTH, which can be indistinguishable from GCT. Laboratory analysis (Ca, ph,
& PTH levels) should be performed to exclude this possibility
Staging,

JAFFE HISTOLOGIC STAGING:

Grade

Behavior

MnGC

GI
G II
G III

Benign PXR
Aggressive PXR
Metastasis

15
70
15

Mononuclear Mitotic Figures

z & small

z
Numerous
y

Absent
Moderate + Atypia
Marked atypia + pleomorphism

CAMPANACCI RADIOGRAPHIC GRADING SYSTEM

Grade

Behavior

Margin

Cortex

GI
G II
G III

Latent
Active
Aggressive

WELL DEFINED
RELATIVELY WELL DEFINED, NOT SCLEROTIC
ILL DEFINED

Intact
Thin expanded
Eroded

No correlation exists bet. grading systems and the incidence of recurrence or metastases
DNA analysis has shown only limited value in prediction the behavior of GCT
Recent studies with CT & MRI improved evaluation of soft tissue extension more

reliable data for staging GCT and predicting their clinical behavior
PXR

GEOGRAPHIC BONE LYSIS + narrow zone of transition + z surrounding sclerosis (80%)

Geographic aggressive growth + wide zone of transition (20%)

ECCENTRIC EPIPHYSEAL ...............................(90%); but large lesions appear central at presentation

Cortex THINNING, EXPANSILE remodeling ........(60%)
Cortical PENETRATION ...............................(50%) often an associated soft-tissue mass
OPERCULUM on metaphyseal end ..............is a medullary plug of compressed bone
Prominent TRABECULATION .......................(50%) MULTILOCULATED = SOAP BUBBLE appearance

pseudo-trabeculation from osseous ridges created by endosteal

scalloping.
This PSEUDO-TRABECULATION is well seen by comparing PXR CT
Pathologic fracture ...................................(30%)
Periosteal reaction ....................................(20%)
Sclerotic rim .............................................(2%)
Multicentric locations ..............................(< 1%) = involve both sides
of a joint:
More common in atypical sites.
More common among young patients (80% < 25 years old).

Bone Scan

y Tc
static uptake in the vast majority of GCT
y uptake peripherally photopenia centrally DONUT SIGN (60%)
99m
Blood pool imaging, dynamic Tc
scintigraphy & Ga67 imaging reveal y uptake; but less
99m

than delayed (static) bone scintigraphy

90
Angiography
Angiography of GCT is only infrequently performed since the advent of CT and MRI:

Hyper vascular .......................... (60%)

Hypo vascular ........................... (30%)
Avascular .................................. (10%)
Preop transcatheter arterial embolization can be used to z blood loss during surgery
CT
CT improves detection of .......................... cortical thinning, expansion, path #, periosteal reaction
CT also helps confirm........................... absence of mineral,

although callus related to pathologic # healing may be seen

ABC component detection.................... FLUID-FLUID LEVEL
MRI

NB

ABC is well seen CT & MRI

(better); as multiple fluid level in
sagittal & axial cuts
Wait 10 min before taking graphs
allowing fluid to sediment

ABC components in GCT..................... 14% (relatively common)

GCT + 2ry ABC ................................... 40% (the commonest lesion to have 2ry ABC)
GCT + ABC ......................................... more aggressive PXR; expansile cystic component
ABC component detection.................... FLUID-FLUID LEVEL
CT is superior to MRI in periosteal reaction, pathologic #, and absence mineralization.
MRI superior to CT in delineating soft-tissue tumor extent

Treatment

1- BENIGN GCT
Depends on:
1. Location of the tumour.
2. Size of the tumour.
3. Tumour grading.
4. Soft tissue extension & articular involvement Joint preservation is impossible
Benign GCT
Excision
Alone

Curettage

Reconstruction
Prosthetic

Bone Graft

Bone Cement

Biological

Distraction

BG

Curettage
Intralesional EXTENDED CURETTE is a limb-sparing option that offers good functional outcomes
Simple curettage BG ......................... RECURRENCE rates of 30-50%
The entire tumour cavity....................... must be adequately exposed for thorough curettage
A large CORTICAL WINDOW ..................... necessary to expose the entire tumour
The intraosseous tumour bulk is removed with a large curette till normal-appearing bone
The cavity is then enlarged ................... HIGH-SPEED BURR (5mm) in all directions + ADJUVANT
High speed burr
Not only MECHANICALLY REMOVE remnants; but also adds a THERMAL NECROSIS for the tumour
Burring of the cavity then

FollowedBy

chemical or physical ADJUVANT + PMMA or BG

91

Local adjuvant agents

1. Physical adjuvant Liquid Nitrogen (LN)
2. Chemical adjuvant Phenol
z local recurrence rate (From 50% 10%).
Liquid Nitrogen
Cryotherapy is the therapeutic use of cold (LN) to induce tissue necrosis.
Mechanism of necrosis via:
Formation of intracellular ice crystals
Membrane disruption
Electrolyte changes
Denaturation of cellular proteins
Microvascular failure.
After curettage LN is poured through a stainless steel funnel left till evaporates
The surrounding tissues are irrigated with warm saline to prevent thermal injury
The process is repeated 2-3 times, resulting in cellular death at a depth of about 1-2 cm.
Fracture is the most commonly reported complication.
Recurrence rates 2-12%
Phenol
Phenolization is an effective and safe local adjuvant therapy for GCT.
Mechanism: Phenol (5%) causes protein coagulation, DNA damage and tissue necrosis.
Technique: Phenol is simply applied by using soaked swabs remaining phenol is sucked
cavity is filled absolute alcohol irrigate with isotonic saline
Advantage:
Compared LN2 it has reduced penetration power = 1-1.5 mm of bone necrosis z fracture.
Reconstruction of defects after curettage and adjuvant treatment is done using either:

Autologus bone graft with or without allograft

Bone cement
Combined (bone Graft + bone cement)
Curettage and bone grafting:
After intralesional curettage high speed burr adjuvant pack the lesion autograft
allograft to fill the defect completely
When necessary, ORIF is used to buttress the bone
Advantages:

Restoring normal biomechanics to the joint surface to z OA

Restoring bone stock, which may help if future procedures

Disadvantages:

The limb must be protected for along time to prevent a pathologic #.

Tumor recurrence is difficult or impossible to distinguish from graft resorption.

92

Curettage and bone cement:

Intralesional curettage followed by packing cement z recurrence than that BG.
Mechanism of action:
Direct cytotoxic effect on the tumour cells
The thermal effect of the polymerization reaction 2 - 3 mm of necrosis
Can stand high stresses so it is preferred more than BG. In knee lesions because
defects in these areas usually too large to be filled BG & to heal out collapse
Advantages:
Lack of donor-site morbidity.
An unlimited supply.
Immediate structural stability.
Low cost.
Ease of use
Radiopaque cement sharply contrasts bone, so recurrence is more readily detected
Disadvantages:
Difficulty in removing it when revision is needed.
Subchondral cement may predispose to early OA.
Excision:
Indicated only for
Grade III tumours.
Displaced pathologic # + poor bone stock not allowing for ORIF cement
Joint destruction
Biological reconstruction using bone graft can achieve:
1. Joint mobility as by using osteochondral proximal fibular autograft or allograft in
cases of GCT of the distal radius and proximal humerus.
2. Joint fusion as by using iliac crest, fibular graft or rotational graft
2- RECURRENT BENIGN GCT
The most important factor for prognosis is the ADEQUACY OF TUMOUR RESECTION.
Recurrence after curettage > wide excision.
Jaffe & Campanacci grading are unreliable
Time: most recurrences are expected in 2y (some pts remain at risk up to 30y)
Pathology: Recurrent GCT usually has malignant biological behavior.
Treatment:
Repeat curette: If benign behavior & no soft tissue extension & no articular damage
Wide excision. If pathological # / soft tissue extension / articular damage
Radiotherapy is recommended when:
Complete excision or curettage is impractical surgically (spine and sacrum)
Medical contraindication for surgery
Aggressive multiply recurrent GCT of bone
Amputation is indicated in massive local recurrence beyond reconstruction.

93

3- BENIGN GCT WITH PULMONARY METASTASIS

Although GCT is typically benign, lung metastases occur in 2 - 6 % of cases.
Primary lesion & metastasis usually have the same HISTOLOGICAL FEATURES of a benign tr
Pulmonary metastases may be MULTIPLE.
High risk for development of metastasis:
Recurrent lesions
Radiologically stage III lesions
Vascular invasion by tumour growth or surgical manipulations
Pulmonary metastases are divided into 3 types:
1. The spontaneous regression type
2. The continuously slow-growing type
3. The rapid-growing type.
About 20% of the patients with continuously slow-growing type and rapid-growing type die
of the disease.
Survival of the remaining 80% suggests that benign GCT of bone with pulmonary metastasis
has a relatively good prognosis
Solitary metastasis resection + ttt of the primary lesion
When pulmonary metastases cannot be surgically excised local DXT + chemo

4- MALIGNANT GCT
Malignant giant cell tumour is a term used to describe a heterogeneous group of giant cellcontaining lesions that are capable of MALIGNANT BEHAVIOR and PULMONARY METASTASES.
Prevalence: .......................................... 5%-10% of all GCT
Site: ....................................................... distal tibia and sacrum.
Types:
Primary malignant GCT (de novo lesions)
Extremely rare
Occur most frequently in recurrent cases.
Secondary malignant GCT: (Radiation sarcoma)
Are sarcomas that occur at the sites of
previously treated GCT by DXT
Treatment:
Surgery alone
Surgery and chemotherapy.
Radiotherapy alone

`|vxtx
o ADAMANTINOMA
o CHORDOMA
o HEMANGIOMA

97

Adamantinoma
An EXTREMELY RARE, locally aggressive, osteolytic, LG malignant tumour of epithelial origin of long
bones. It is not related to adamantinoma or ameloblastoma of the mandible and maxilla which is
derived from Rathke's pouch
Incidence

Age ....................................... 10-40 Y

M>F

Pathology

Osteofibrous dysplasia (OSSIFYING FIBROMA) has a striking predilection for the tibia and has
well documented association with adamantinoma and may be a benign precursor to it
Site: tibia .............................(90%)
Gross:
o GRAYISH white RUBBERY mass areas of HGE and necrosis
o Bone SPICULES and CYSTS filled blood or straw-colored fluid
Microscopic
o Biphasic tumor...........epithelioid cell islands + surrounding reactive fibrous stroma
o The stroma consists of spindle cells producing collagen

Presentation

History of TRAUMA usually associated with adamantinoma (remains unclear role)

The patient usually has SWELLING that may be painful of 3WK-3Y duration

Radiology

ECCENTRIC, well-CIRCUMSCRIBED, LYTIC lesion + cortical THINNING + LITTLE PERIOSTEAL REACTION

Several lytic defects separated by sclerotic bone "SOAP-BUBBLE" appearance.

The lesion may break through the cortex and extend into soft tissue
MRI helps demonstrate the intraosseous and extraosseous involvement.

Differential diagnosis (radiologically)

Osteofibrous dysplasia
fibrous dysplasia
ABC
chondromyxoid fibroma
chondrosarcoma.

Treatment

WIDE SURGICAL EXCISION

RADIO & CHEMO-RESISTANT

Prognosis

May METASTASIZE to Lungs, LN, Liver by both haematogenous and lymphatic routes
Survival metastasis............. 12 Y
Local recurrence ................... 20%

98

Chordoma

RARE malignant tumor arises from remnants of notochord!!

Incidence:

Age .....................................................40-70Y
M:F .....................................................2:1

Aetiology:

At 4-6 wk of fetal development, mesenchymal cells from sclerotomes

merge to surround the notochord & form vertebral bodies.
The notochord normally degenerates Re
mnantsForm

nucleus pulposus
The prevailing theory is that in chordomas the notochord fails to
degenerate and then undergoes malignant transformation.
Against this; normal notochord remnants have never been observed!

Pathology

Sites:
o
o
o
o

Gross
o
o
o

SACROCOCCYGEAL region............50%
SKULL BASE.................................30%

Transverse processes of vertebrae

Paranasal sinuses.

SOFT, BLUE-GRAY, LOBULATED, ENCAPSULATED tumor

GELATINOUS translucent areas
Tumor TRACKS ALONG NERVE root at sacral plexus

or sciatic notch in planes of least resistance

Microscopically:
o LOBULES + FIBROUS SEPTA.
o "PHYSALIPHOROUS" cells: EOSINOPHILIC CYTOPLASM + MUCIN VACUOLES push the nuclei to
the side resulting (=greek word for bubble)

Clincal

Sacrococcygeal tumours:
o Low back PAIN (no characteristic pattern or course)
o Bowel and bladder DYSFUNCTION
o Often large at presentation & can be palpated on PR
Other sites:
o DYSPHAGIA .................................Anterior cervical tumours
o NEUROLOGICAL DEFICITS ..............Posterior cervical tumors
o HEADACHE .................................Base of the skull

Radiology:

PXR: solitary mid-line LYTIC lesion + bony DESTRUCTION + soft tissue MASS + focal CALCIFICATION
CT & MRI: demonstrate soft tissue + epidural extension + calcification + identify recurrence
Chordomas have reduced uptake on bone scan.

Treatment

WIDE EXCISION ........................................... (rarely feasible the anatomic location of the tumor)
Sacrococcygeal tumor............................ sexual & sphincter dysfunction after surgery
RADIATION ................................................. if complete resection is impossible
CHEMOTHERAPY ........................................ for late stage disease.

Prognosis:

Chordomas METASTASIZE to lymph nodes, lungs, liver and bone.

99

Haemangioma Of Bone
Features

Site

Haemangiomas are hamartomas characterised by vascular spaces lined with endothelial cells
Common, ~ 10% of autopsy cases having vertebral haemangiomas
M:F .....................................................1:2
Age......................................................30 - 50 Y
VERTEBRAL BODIES (thoracic) ................50%

Calvarium............................................20%
Also tibia, femur and humerus

Pathology

GROSS: Vascular HAMARTOMA with CYSTIC, DARK RED CAVITIES (DDx: ABC, telangiectatic OS)
4 types:
o Capillary
Most Common
o Cavernous
o Arteriovenous: remnants of foetal capillary beds
o Venous
MICROSCOPY : Non-vascular components: fat, smooth ms, fibrous, bone, haemosiderin & thrombus

Presentation

Usually asymptomatic and solitary.....discovered on x-ray or at post mortem

Vertebral haemangiomas ....................chronic back ACHE + neurological symptoms
Pathological .......................................FRACTURE
Long bones may .................................OVER GROW y blood supply

Radiology

PXR

Vertebral lesions .................................coarse, thickened vertebral trabeculae erosion of the

horizontal trabeculae CORDUROY appearance
Vertical striations without bone expansion (DD Paget's)

Body lesions .......................................POLKA DOT as the vessels are seen in cross section
Calvarial lesions .................................lytic radiating WHEEL SPOKES
Metaphyseal or epiphyseal .................lytic lesions spiculated pattern of "IRISH LACE"

CT

MRI

T1 vary from low to high intensity depending on fat content

T2 sequences demonstrate lesions with high signal due to the vascularity

Treatment

Asymptomatic.....................................No ttt
calvarium Lesions ...............................resected thin margin of normal bone
Vertebral lesions .................................radiation or excision preceded by embolisation
Metaphyseal lesions ...........................excision + BG

100

High Grade Angiosarcoma of Bone

Seems to have two distinct clinical presentations:

o First, the lesion can present as multiple lesions in a single bone, two or more adjacent
bones, or perhaps all the bones of a limb. These lesions seem to have an indolent
course and the prognosis remains good.
o The second presentation is that of single or multiple rapidly progressive lesions that
metastasize to other bones or to the lung this form of the disease has a very poor
prognosis.

fy g|x ftvt
o PERIPHERAL NERVE TUMOURS
o SYNOVIAL TUMOURS
o FIBROUS TUMOURS
o MUSCLE TUMOURS
o VASCULAR TUMOURS
o FATTY TUMOURS
o RARE SARCOMAS
o BONE METASTASIS

103

Soft Tissue Tumours

Incidence
Peripheral Nerve Tumours
Vascular Tumours

Staging
Synovial Tumours
Fatty Tumours

Diagnostic Clues
Fibrous Tumours
Rare Sarcomas

INCIDENCE
Benign soft tissue tumours common
Malignant soft tissue tumours rare
Pathological Classification:
1. Benign
2. Malignant
3. Reactive tumour like lesions
Tissue of origin
Benign
Malignant

Diagnostic Steps
Muscle Tumours

Reactive lesions

104

DIAGNOSTIC CLUES
Size
A small mass < 5 CM in its greatest dr ............unlikely to be malignant
Mass that is > 5 cm ........................................20% chance of being a soft tissue sarcoma
The size of the lesion can be determined by physical examination if the lesion is
subcutaneous and easily palpable, or by ultrasound, computed tomography (CT) or magnetic
resonance imaging (MRI).
Site: Superficial or deep?
Superficial lesions are more likely.................. benign or malignant better prognosis than deep
The depth is best determined by ....................physical exam, ultrasound or MRI.
The THIGH and BUTTOCKS are the two most common sites for soft tissue sarcomas. Any large
deep mass in the thigh or buttocks should be considered at high risk for being a sarcoma
Consistency
Soft tissue sarcomas tend to be ...................... FIRM and NOT VERY PAINFUL until they get very
large and compromise their vascular supply or adjacent neural structures.
Lipomas are usually ....................................... SOFT and NON TENDER
Infectious and inflammatory .......................... WARM and TENDER
Pseudoaneurysm............................................. PULSATILE AUDIBLE BRUIT on auscultation.
Cystic or solid
Most cystic lesions are .................................... INFLAMMATORY or BENIGN, as ganglion & abscess
Solid lesions is either ...................................... benign or malignant neoplasm.
Attempt Transillumination
If deep ultrasound or MR scan will determine this
Duration
A mass that has rapidly increased in size over two months is more likely to be a sarcoma
than the lesion that has slowly enlarged over a 20-year period.
A mass that increases and decreases in size is usually a cystic lesion.
However, caution should be taken with masses that have been present for a long time. Soft
tissue sarcomas occasionally present with a history of many years duration up to 30 years.
PXR
Every soft tissue mass that is going to undergo intervention should have a plain radiograph
Fat density lesion............................................. lipoma
Punctate calcifications..................................... synovioma, chondrosarcoma, hemangioma
Ossification...................................................... osteosarcoma or myositis ossificans
skeletal abnormalities...................................... osteomyelitis, primary bone lesion or periosteal
reaction from the soft tissue tumor
MRI
The MRI gives the most information of any radiographic study but should be reserved for
large lesions or those that are ill defined.
It will clearly delineate whether the lesion is a bone lesion with a very large soft tissue
component (bone malignancy) or whether the lesion is a primary soft tissue lesion.
z Tl & T2 .....................................................either an extra-abdominal desmoid tumor,
extensive scar tissue, cortical or dense bone or a foreign material such as bone cement or air.
y T1 & T2 .....................................................likely a lipoma.
z T1 & T2 ....................................................low-grade liposarcoma & any neoplastic lesion;
benign or malignant.

105

DIAGNOSTIC STEPS
History
Examination +/- transillumination
Ultrasound and plain radiography
If lesion< 5cm, cystic and subcutaneous ........... observe
(If patient keen on removal use a longitudinal incision with good haemostasis, ensure the
incision can be encorporated in any later excision and perform an excisional biopsy)
If lesion >5cm, not cystic, or painful.................. MRI, then incisional biopsy / trucut needle
biopsy / fine needle aspiration (see principles of biopsy)
99m
If a possibility of malignancy ............................. Tc
, CXR, Chest CT, Abd CT, CBC, ESR

TREATMENT
Soft tissue sarcoma treatment ............................ resection, amputation, DXRT or a combined
Systemic control includes ................................... chemotherapy is controversial because it has
not been very effective in improving survival & has significant patient morbidity & cost.

PROGNOSTIC FACTORS
1. Stage of the patient (Ennekings stage)
o Metastasis is most frequently identified in the lungs LN skeleton.
o The standard staging studies include a physical examination of LN, CXR, CCT, Tc,
and gallium scan.
o A patient non-metastatic disease at presentation has a far better prognosis
2. Histologic grade.
o Patients HG lesions have a worse prognosis than patients LG lesions.
3. Size of the lesion
o Although small lesions (< 5 cm) are rarely malignant, when they are, they have a
better prognosis than larger lesions.
4. Depth of the lesion
o Superficial (subcutaneous) soft tissue sarcomas have a better prognosis than deep
(below the muscle fascia) lesions.
TIOLOGY:
1. Genetic: e.g. mutations, translocations, deletions, monosomy, trisomy as in NF1
2. Radiation: induce mutations
3. Carcinogenic: e.g. thorium, vinyl chloride
4. Lymphedema: predispose to lymphangiosarcoma
5. Infection: HPV 8, Epstein-Barr virus
6. Trauma

106

Benign Peripheral Nerve Tumours

1. Neurilemmoma (benign schwannoma)
BENIGN nerve sheath tumour
YOUNG to middle aged patients
Usually ASYMPTOMATIC apart from the mass
MR scan shows eccentric MASS arising from a peripheral nerve
Histologically:
o ANTONI A- compact spindle cells, twisted nuclei, indistinct cytoplasm, clear vacuoles
o ANTONI B-less cellular
TREAT ..........................excision leaving the nerve intact
2. Neurofibroma & Neurofibromatosis (see neurofibromatosis)
CP: ............................Most are SUPERFICIAL, grow SLOWLY and are PAINLESS
Gross: .........................Expand the nerve in a FUSIFORM fashion
Mic: ...........................interlacing bundles of ELONGATED cells with WAVY DARK nuclei
Treatment ...................excision with a marginal margin
malignant change .......In neurofibromatosis (5-30%)
3.MORTONS NEUROMA
Interdigital neuroma was first described in 1845 by Durlacher, chiropodist to the Queen of England, as a
"form of neuralgic affection" involving the plantar n. between 3rd & 4th MT bones. In 1876 Morton
expanded the clinical description and postulated that the neuroma resulted from pinching of the common
digital branch of lateral plantar n. to 4th web space between the mobile 4th & 5th MT heads. Other theories:
Anatomy
th
4 digital br. of medial plantar n. emerges from beneath the medial side of FDB and while coursing
obliquely across the plantar surface of the muscle receives a communicating branch from the
common digital br. of lat.plantar n. The "neuroma" (more precisely degenerative & fibrotic

changes) occurs in the common digital n. near its bifurcation.

Aetiology A number of causative factors have been suggested:
1. Tethering of the 3rd space n. by the ANASTOMOTIC BR. between medial and lateral plantar nn.
2. Tethering of the n. by FORCED TOE DOSIFLEXION in high-heel shoe/hindfoot VALGUS/interdigital BURSITIS
3. LAXITY of transverse MT lig. plantar displacement of MT heads pressure on adjacent digital n.
4. Relative NARROW 3RD INTER MT space
5. Relative HYPERMOBILITY of lateral 2rays (joined to cuboid), on med. 3rays (joined to MT cuneiform)
6. INSTABILITY of the 4th MTP joint
7. Pressure on the nerve during BW PRESSURE NEURALGIA
8. TUMOR involving the lateral most branch of the medial plantar n.
9. LUMEN OCCLUSION in the common digital A. adjacent to the n.
rd
10. DEGENRATION OF MTP3 deviation of the 3 toe medially pushing the MT3 laterally impenging the n.

107
11. Pathological Findings
HISTOLOGICALLY:

1.
2.
3.
4.
5.
6.

Perineural fibrosis
Thickened vasanervosa hyalinized walls
Neuronal edema
Neuronal demyelinization and degeneration
No inflammatory changes
Frequent presence of bursal tissue

ELECTRON MICROSCOPY:

y in the normal Amorphous deposition & y eosinophilic tubular filaments

In a strict sense the term neuroma is not correct because the haphazard proliferation of axons seen in
a traumatic neuroma is not found, and the deposition of hyaline and collagen filaments accounts for
the enlargement is probably degenerative rather than proliferative, with repetitive trauma against the
deep transverse intermetatarsal ligament being the most likely cause. Repetitive microtrauma,
perineural fibrosis, vasa nervorum occlusion, and endoneural edema probably are causations
Clinically:

Neuralgic PAIN:
rd
nd
th
o Plantar pain at 3 interdigital space (some times 2 but 4 is rare)
o y walking
o y at night sometimes
o z on removing shoes
TINGLING, NUMB, BURNING, or "DEAD" toe
Vague FOREFOOT tingling
COLOUR changes
The condition may remain undiagnosed for many years.

Clinical assessment

The diagnosis is often strongly suspected within the first minute of the consultation.
Ask about:
PN ....................................................DM and chronic inflammatory disorders
Foot Trauma
Ankle Discomfort ............................may suggest tarsal tunnel syndrome
Spinal problems ...............................history of root entrapment symptoms.
Examination
Any nerve entrapment in spine, proximal limb or tarsal tunnel.
On local examination look for:
o Local TENDERNESS
o SWELLING in the intermetatarsal space
o MULDER'S CLICK or crunching sensation + pain
o LOCAL ANAESTHETIC injection into the affected space
o whole foot should be examined for any cause of METATARSALGIA

A, Squeezing forefoot just proximal to metatarsal heads between index finger and thumb
B, Simultaneous compression of the suspected web space two fingers of opposite hand

Imaging

US & MRI have been described for imaging a neuroma, but we have not had any success
If there is a suggestion of other forefoot pathology PXR should be obtained.

108
Treatment:

NON OPERATIVE TREATMENT:

1.
2.
3.
4.
5.

Metatarsal bars or pads

Local injection of a steroid preparation into the affected web space,
wide toe-box shoes
Avoid high heel
No place for orthosis

OPERATIVE TREATMENT:

The mainstay of treatment, of course, is surgery 90% satisfaction

Division of deep transverse inter MT lig is controversial may lead to dropped MT, but is
needed in the dorsal approach
Painful scar may result from plantar incision despite its better visualization out MT lig cut
Never do a transverse plantar incision
Plantar approach is better in the case of recurrent cases; but avoid superficial dissection for
fear of fat pad necrosis and painful scar
The nerve is divided 2-3cm proximal to the bifurcation and excised
Decompression of the interdigital space with excision of the bursa
Warn patients that it may take several months to reach full benefit & that they may develop a
new neuroma on the severed nerve end which may be more painful than the original one
DORSAL APPROACH

The dorsal approach is the most common and widely used surgical treatment for interdigital
neuroma. Overall, one can expect 90% good and excellent results with this approach
Under tourniquet, make 3 cm incision starting in the involved web space (usually 3rd)
Place the incision between the metatarsals to avoid injury to a small dorsal sensory nerve
Carry out blunt dissection with a thumb or small scissors down between the MTs
Place a retractor or a small lamina spreader into the wound between the metatarsal heads,
and open it, placing the transverse metatarsal ligament under tension
Use a Freer elevator to bluntly dissect in line with the incision to identify the transverse
metatarsal ligament and the normal interdigital nerve proximally. The nerve occasionally
courses close to the metatarsal and is not always in the center of the interspace .
Plantar pressure under the web space should reveal the enlarged neuroma protruding distal to
the transverse metatarsal ligament . Then sharply divide the metatarsal ligament.
Identify the nerve in the proximal part of the wound and trace it distally into the irregular
mass. Look for any accessory branches coming from either metatarsal to join the common
digital nerve. If one is identified, dissect it and clearly define it. This prevents the nerve end
from retracting & forming a potential painful neuroma stump directly under metatarsal head .
Trace the common digital n. and transect it in the most proximal aspect of the wound so the
cut end does not end up bet metatarsal heads. Then dissect neuroma out distally and excise
PLANTAR APPROACH

Palpate the metatarsal heads on each side of the involved interspace and draw them
Place a small-gauge needle (25-27 gauge) from a dorsal to plantar direction in the interspace,
piercing the plantar aspect of the foot. This identifies the interspace plantarward so the
incision can be made accurately. Draw the incision in line interspace, but proximal
Carry the incision through the subcutaneous tissue. Place a Weitlander retractor. Carryout
dissection with a blunt scissors in line with the skin incision. Retract slips of the plantar
fascia, exposing the interdigital nerve and neuroma. Transect. the nerve proximally and
continue dissection distal to the neuroma. Excise the neuroma as in thedorsal approach

109

Malignant Peripheral Nerve Tumours

1. Neurofibrosarcoma
Rare
Can arise de novo or in neurofibromatosis
Tend to be high grade, therefore treated with wide surgical resection +/- radiotherapy

Benign Tumors of Muscles

Leiomyoma, Rhabdomyoma (glycogen tumor)

Seldom occur in the extremities

Malignant Tumors of Muscles

1. Leiomyosarcoma
HG or LG tumor arising from the smooth ms of blood vessels
Gross: Appear encapsulated but avoid mere enucleation of the tumor
Mic: Tumor cells elongated nuclei mitotic figures & sometimes arrange in palisades
Wide/radical surgical excision and radiotherapy
2. Rhabdomyosarcoma
The MOST COMMON sarcoma in young patients
Among the MOST MALIGNANT sarcomas; grows rapidly, yyy metastasis to LN
Microscopically:
Type
Embryonal:
Boytroid
Alveolar:
Pleomorphic

Age

Site

Adolescent

Limb & head

Principle cell

spindle cells
(ycytoplasm)
Embryonal type but occur in UT beneath epithelium
Adolescent
Limb & head
round cells
(zcytoplasm)
Adult
UL & LL
Spindle cells

MnGC Special feature

+

y Myxoid areas

Rhabdomyoblasts
( striations)
Racquet cells

sensitive to multiagent chemotherapy

Treat with preop chemo, followed by wide surgical excision and radiotherapy

110

Synovial Tumours
A. Benign tumors
l. Tenosynovial giant cell tumor
a. Localized tenosynovial giant cell tumor
b. Diffuse tenosynovial GCT (extraarticular PVN synovitis, florid tenosynovitis)
2. Ganglion
3. Synovial chondromatosis
4. Tumoral calcinosis
B. Malignant tumors Synovial sarcoma
1. Biphasic (fibrous and epithelial) synovial sarcoma
2. Monophasic (fibrous or epithelial) synovial sarcoma

Ganglion Cyst
Ganglia are the most common cause of focal masses in the hand and characteristically arise either
from synovium of joints or tendon sheaths or from tendons, where they may cause trigger fingers.
Incidence:

Ganglion cysts are the most common soft tissue tumors of the hand and wrist.
2ND & 4TH decade ...................................... 70% (Any age, including children)
: ............................................................. 3:1
No relation to side or occupation
Etiology theories

1- MUCOID DEGENERATION:

The most accepted explanation ............ MUCOID DEGENERATION of collagen and CT.

2- TRAUMA AND TISSUE IRRETATION:

A more recent theory ............................ TRAUMA or IRRITATION Synovial cells to produce

mucin sneak its way out across capsule form a duct function as valve like producing
lakes. Mucin ducts and lakes eventually coalesce to form a solitary cyst

3-DEFECT IN CAPSULE OR TENDON SHEATH:

Such defect allow protrusion of synovial tissue

Pathology:

Site:
Dorsal wrist ganglions ................... 70% (scapho-lunate lig bet 2nd & 4th ext tendon comp)
Volar wrist ganglions ................. 20% (scapho-trapezial joint just radial to FCR tendon)
Flexor tendon sheath ..................... 10% (at the level of the A1 pulley)
Gross:
Well CIRCUMSCRIBED, SMOOTH-walled, WHITE and TRANSLUCENT mucin filled cysts
Ganglion cysts may be single or multilobulated (communicate via duct network)
Ganglions are usually CONNECTED by a stalk to an underlying joint capsule or ligament.
Ultrastructure:
Highly viscous mucin................. HYALURONIC + ALBUMIN + GLOBULIN + GLUCOSAMINE
Wall is made up of ..................... COLLAGEN out epithelium, synovium, nor necrosis

111
Clinical Picture: ........................................... ASYMPTOMATIC

Presenting symptoms................................. PAIN, z MOTION, PARESTHESIAS, and weakness .

Ganglions are usually ................................ SOLITARY & < 2 CM in diameter.
Ganglia occasionally.................................. disappear spontaneously
Sometimes RECUR when treated by........... rupture or aspiration

DIP GANGLION = mucous cyst

Arise dorsally bet DIP JOINT crease and eponychium (on either sides of the extensor tendon) occur

in OA of DIP & may lead to longitudinal grooving of the nail plate long pressure on the
germinal matrix
OCCULT DORSAL GANGLION:
SCAPHOLUNATE tenderness extreme wrist motion, especially in extension. Radiographic findings

are often normal, and MRI is useful in confirming the diagnosis. Surgical excision of the occult
ganglion is successful for alleviating pain and symptoms in the majority of cases.
Investigations

PXR (cyst is not seen) ............................... evaluate any bone or joint abnormality
MRI ............................................................ confirm atypical presentation e.g. occult ganglia
ALLEN TEST ................................................. evaluate the blood flow to the hand
TREATMENT

NON SURGICAL THERAPY

Aspiration steroid injection. This is especially successful for tendon sheath ganglions in the hand
and digits. Avoid multiple steroid injections to avoid skin and fat atrophy & hypopigmentation.
SURGICAL THERAPY:

Generally .................................................... Total ganglionectomy + portion of the capsule

Dorsal wrist ganglion:
GANGLIONECTOMY + attachment to SCAPHOLUNATE ligament
Usually approached through a transverse incision
Volar carpal ganglion:
GANGLIONECTOMY + attachment to radio-carpal capsule of the SCAPHO-TRAPEZIAL joint
Longitudinal incision
Volar retinacular ganglion (A1 pulley)
Transverse incision near the distal palmar crease.
Other ganglia of the volar digits:
GANGLIONECTOMY + small portion TENOSYNOVECTOMY
Via Bruner or mid-lateral incision identify the bundle
Mucous cyst:
Curved oblique incision, or an H-shaped incision, on dorsal DIP
If the overlying skin is adherent it is excised in an elliptical fashion
Cyst is dissected proximally to the DIP GANGLIONECTOMY + PARTIAL DIP CAPSULECTOMY
Surgical excision of a ganglion should include the removal of a generous capsular margin about
the cyst base, and no attempt should be made to close the joint capsule
Recurrence after complete excision is rare
Arthroscopic resection of dorsal wrist ganglions is currently being investigated.

112

Pigmented Villo-Nodular Synovitis

Introduction:

Benign Synovioma = GCT of Tendon Sheath

xanthomatous non neoplastic lesion affecting synovium of joints and tendons, characterized by
deposition of hemosidrin and cholesterol. It occurs due to idiopathic synovitis.
Epidemiology

2/106/y
Age ......................................................... 20-45 y (range 11-70)
=........................................................ equal
No environmental, genetic, ethnic or occupational predilection .
Since bursae & tendon sheaths are related to synovium, they may have xanthomatous growths
Commonest presentation ....................... monoarticular pain and swelling .
Sometimes .............................................. polyarticular involvement

Pathogensis Theories:
1.
2.
3.

PROLIFERATIVE REACTION ......................... unknown inflammatory or antigenic agent

NEOPLASTIC TRANSITION ............................ from the inflammatory to proliferative "tumour"
REACTION TO INTER-CELLULAR HGE ............ attractive hypothesis as MnGC / xanthoid cell /

hemosiderin are often a prominent feature, all of which factors are caused by extravasated
blood. But the lesion is not common in hemophilics who commonly bleed into their joints &
also the lesion can't be produced by injecting blood into the joint cavity.
4. Recently; ALLERGIC INFLAMMATORY REACTION to hemosiderin or its metabolites
Pathology:
SITE:

1. Related to tendons: (Giant cell tumour of tendon sheath).

Common ...................................... FLEXOR TENDONS SHEATH of the hand
Less common .............................. EXTENSOR ASPECT OF IP joint (often the P.I.P joint).
Rarely .......................................... ankle & the toes are involved.
2. Within joints: Pigmented Villo-Nodular Synovitis (PVNS)
most commonly........................... KNEE (80%)
Other joints.................................. hip, ankle, shoulder, wrist
3. Rare in bursae
4. Recently described in cancellous bone (Intra-osseous PVNS) that occur after a long standing
articular PVNS that followed by erosion of the bone, and the synovial tissue penetrates into the
underlying cancellous tissue & continues to proliferate there.
MACROSCOPICALLY:
1. PIGMENTED VILLO-NODULAR SYNOVITIS:

Localized or diffuse form.

The localized form ...................... identical histologically AS GCT
The diffuse form.......................... same but involve the ENTIRE KNEE SYNOVIUM
May produce a BULKY mass......... Gives false impression as if it is a sarcoma
Tumor may cause BONE EROSION Gives false impression as if it is a sarcoma
Tumor may break through the articular plate & invade the metaphysis & in rare
instances no perforation can be found, as if the metaphysis has the same pathology
2. GIANT CELL TUMOUR OF TENDON SHEATH:
PEA-SIZED, bright YELLOW, LOBULATED, ENCAPSULATED, firmly ATTACHED to its origin
STREAKS of yellow & brown of hemosiderin & cholesterol ester
Sometimes PROJECTIONS arise from the mass & dissect between tendons recurrence

113
MICROSCOPICALLY:
1. IN THE EARLY STAGE:

Non-specific inflammation ......... thick edematous synovium

HEMOSIDERIN deposition............... RED-BROWN colour.
SHAGGY or BEARD appearance...... fibroblastic reaction & villous reaction
LYMPHOCYTIC infiltration
2. DOMINANT PROLIFERATIVE REACTION BECOMES MORE:

Nodules of solid tissue are produced.

3. EVENTUALLY:

Very cellular sheets of ................ SPINDLE, cylindrical, or POLYHEDRAL cells

Fibroblastic & synovial .............. HYPERPLASIA
SYNOVIAL LINED CLEFTS .................. suggesting the synovial origin of its cells.
MNGC formed by ......................... fusion of contiguous lining cells.
LIPID-BEARING HISTIOCYTES ............ are found scattered in FIBROUS STROMA.
XANTHOID CELLS ........................... are found scattered in FIBROUS STROMA
Stromal cells may appear ...........undifferentiated ConfuseWit
h sarcoma & GCT
Cellularity
PVN
GCT

Fibrosis

Pigment

++

Cholesterol

++
++

Diagnos
osis:

A- Pigmented Villo-Nodular Synovitis

Hist:

Vascularity
++

++

Two types:
PVN
1- Diffuse
2- Localized
Age: .... 20 in diffuse
............ 30 in localized
Male & knee are the most common

Gradual onset, intermittent course of moderate PAIN

Associated with ........................... SWELLING & LIMP
Mechanical interference ............. STIFFNESS, locking, snapping & limitation of extension

Exam:
1- Large BOGGY swelling................ in diffuse form

Small palpable JOINT MOUSE ..... in localized form

2- Effusion may be pronounced .... FLOATING PATELLA
3- Generalized TENDERNESS

As
piration:
sp

In diffuse form............................. BLOOD-TINGED fluid y bilirubin & cholesterol

In localized form ......................... effusion is NOT ABUNDANT, STRAW coloured & sterile.
Lab: y S.CHOLESTEROL .............................. High normal normal chol/ester ratio (not diagnostic)
PXR:

NARROW JOINT space

SOFT TISSUE SHADOW .................... thickened synovium
CORTEX EROSION from without..... Superficial or extensive margin of sclerosis

Double contrast arthrogram reveals:

Diffuse form .................. BUBBLY FLOCCULENT effect within the synovial cavity
Localized form............... Soft CREEPING shadow on suprapatellar synovium
CT ............................................................... Best to demonstrate bone involvement
MRI ........................................................... Best to demonstrate soft tissue masses
Arthroscopy:

Arthroscopic examination........... characteristic gross pathological picture of PVN

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B- Giant Cell Tumour of Tendon Sheath

Clinical picture:

Discrete mass(s) fixed to the tendons of the hands & feet & Achilles (and move them)
At first they are PAINFUL .............. later they are PAINLESS
Spontaneous TENDON RUPTURE IS RARE
BILATERAL involvement is usual
DISCRETE, SOFT TO FIRM, PEA-SIZED

Some times ................................. large, bulbous & disfiguraing & they are fixed to &
Overlying skin............................. normal & freely movable over the tumours
Growth is very slow .................... Take years
Yellow plaques around the eye... Part of familial hypercholesterolemic xanthomatosis
Lab:

y s.cholesterol is often associated.

PXR:

Pressure erosion of the adjacent phalanx. The articular surfaces are never involved.
Treatment:

1. PVN
Localized form ............................ MARGINAL EXCISION
Diffuse form ................................ TOTAL SYNOVECTOMY Radiotherapy if surgery fail
If significant bony erosion .......... ARTHROPLASTIC RECONSTRUCTION
2. Giant cell tumour of tendon sheath:
EXCISION, which may be technically difficult in large lesions
Recurrence

Recurrences are not infrequent if the excision is incomplete .

Even after meticulous excision... 27% recurrence rate
Risk factors for recurrence:
Adjacent OA
DIP location
Thumb IP
+ve osseous pressure erosion on PXR
Presence of the dissecting projections if left behind during surgery

115

Tumoral Calcinosis
Definition:

A rare condition, characterized by fine non-tender tumour-like masses

around joints, Ca sallts deposition

Epidemiology

Children
Once occurred other lesions are common to follow!!

Pathogenesis Theories:

Resume
-Child
-Shoulder swelling
-Encapsulated cyst
-Ca salts
-Amorphous & crystals
-MnGC + lymphocytes

Genetic back ground had been proposed

Pathology:
SITE:

Soft tissue around large joints.................Shoulder 60%

Other joints ..............................................elbow and hip

MACROSCOPICALLY:

Encapsulated cystic mass...................... embedded in the collagenous tissues

The cavity of the cyst contain a fluid or semi-f1uid Ca salts.

MICROSCOPICALLY:

Amorphous masses of powdery Ca salts

Mineral crystals
Giant cell reaction about these deposits forming a scalloped & convoluted border
Lymphocytic infiltration & fibrous proliferation

Clinically:

Masses are usually painless

Interfere with function only .................. if develop to a large size
May reach ............................................ 20cm

DD:

1- Calcinosis universalis:
Generalized calcification of skin, s.c. tissues & muscles in children.
2- Calcinosis circumscripta:
Localized deposits of Ca associated with Raynaud's or scleroderma in adults.
Treatment:

Excise lesions that ................................ cause disfigurement or limitation of function

Prognosis And Recurrence

If a solitary lesion of tumoral calcinosis is discovered one can expect that other lesions will
develop in other joints as the patient ages
They are quite apt to recur

116

Synovial Chondromatosis
SYNOVIAL CHONDROMETAPLASIA
Definition:
RARE lesion resulting from cartilaginous or osteocartilaginous

metaplasia in the synovium of joints, bursae or tendon sheaths.

In synovial chondromatosis multiple cartilaginous nodules develop &
grow in the synovium of a joint & may become loose bodies.
Epiid
demiology

Age: ..................................................... 30-50 Y

Sex: ......................................................... MALES predominate

Chondromatosis

-Rare middle aged male

-Metaplasia of
embryonal rests at the
villi top
-Joint Rice
-Snow Storm
-Monoarticular (Knee)
-Locking & Pain (OA)
-+ve PXR only if ossify
-Total synovectomy +
Loose body removal

Pathogenesis Theories:

Considered to arise from ........................Embryonal rests (origin of synovium & cartilage)

1. May represent........................................ detached OCD or traumatic cartilage detachments
2. May represent........................................ detached cap of an INFLAMMATORY SPUR
3. Frequently they result from................... METAPLASTIC ERROR of synovial cells.

Pathology:

SITE:
KNEE, elbow, ankle, hip & shoulder are involved in this order of frequency.
The condition is usually........................ MONOARTICULAR but both knees may be affected.
MACROSCOPICALLY:
HUNDREDS OF SPHEROID CARTILAGINOUS NODULES protrude into the joint cavity
JOINT MICE or RICE BODIES may be formed
Arthroscopically it has a characteristic SNOW STORM appearance
MICROSCOPICALLY:

Hyaline cartilage forms in .................... stratum synoviale at the synovial reflection

Chondroid masses ................................ begin to appear at the tips of the synovial villi
Surrounding cells are altered ................ resemble chondroblasts
Cartilage mass grow & form ................ spheroid body, the villous process being its pedicle
Eventually it becomes detached ........... falls free into the joint space
Chondroblasts continue ........................ Proliferation & production of spheroid cartilage
Chondroblasts may metaplase to .......... osteoblasts nidus of bone surrounded by cartilage
The story ends by.................................. joint distended by uniform glistening white bodies
They cause repeated trauma to the articular surface multiple erosions OA
In the past, this condition had been given a misnomer osteochondromatosis
Diagn
gnosis:
Clinically:

Usually mild, duration long & coarse intermittent.

Pain............................................. dull ache.
Swelling
Stiffness.
Transient locking episodes
Giving way ................................ in case of the knee joint
Signs:
Generalized joint tenderness.
Thickened synovial membrane
Marked audible & palpable crepitus.
Loose bodies may be palpable.

117

Tenosynovial chondrometap1asia:
Sometimes the tendon sheath or bursal synovia may assume the same picture
DDX: .............................................................. CHONDROSARCOMA.
Cartilage SIZES VARY ...............................from microscopic to over a centimeter in diameter.
As they age ............................................their CENTERS MINERALIZE radiopaque.
Sometimes they .................................... LIE in the sheath wall
Other times are found............................as FREE BODIES in the sheath lumen
WRITS & hand.........................................most involved in tenosynovial chondrometaplsia

PXR:

Usually show MULTIPLE LOOSE BODIES (Only when calcify or ossify)

is always much more than seen on PXR
When all are chondromatous ................ air or DOUBLE CONTRAST ARTHROGRAPHY may be
needed
DD:

Monoarticular swelling
Rheumatic arthritis ............................... fragments of articular cartilage detach & incorporate
in the synovium or become free in the joint
Severe OA as multiple osteophytes........ may detach
Treatment:
TOTAL SYNOVECTOMY + REMOVAL OF LOOSE BODIES

Joint mice removed immediately.......... to z damage to articular surfaces

Patient is forewarned ............................ OA is already present residual symptoms
Total synovectomy + All communicating bursae are also excised.
As a general rule the menisci are damaged, requiring removal. The outlook for permanent
cure is excellent.
Prognosis And Recurrence
RECURRENCE is not unusual.

Malignant change very rare.

118

Definiittion:

Malignant Synovial Disorders

Synovioma
Synovial Sarcoma
-32y
-Male

Malignant sarcoma occurs in close proximity to joints but rarely from

an intra-articular lesion. It has adenomatous & fibrous elements

Epidemiology

10% of sarcomas
Age:....................................................... 32y
: ....................................................... 3:2

-Lower limb swelling

-Long History
-Small size
-Adenomtous + Fibrous
-Gray + Ca + pseudocap
-PXR spot calcification

Pathogenesis Theories:

90% translocation ................................. t(X;18)(p11;q11)

Genes affected SSX on Xp & SYT on ch.18 (they are transcription modulators)
Synovial origin is controversial but it is indicated by:
1. Formation of clefts
2. Formation of villous projections
3. Formation of gland like spaces lined by cuboidal cells & filled mucin
4. Formation of cell tufts

Pathology:
SITE:

Most common ...................................... Ankle & foot 80%

Second common.................................... knee
Rarely.................................................... Shoulder, Chest, Abdomen, neck
Usually arise from................................. Tendon sheath & capsule
Only 10% are intra-articular

MACROSCOPICALLY:

Circumscribed, rounded, lobulated, Grayish pink mass

Areas of Ca, cyst, hge, necrosis ........... Brownish color
Compress surroundings......................... pseudo-capsule
Difficult to find synovial attachment

MICROSCOPICALLY:

Difficult cell variations; mainly 3 types

Monophasic
Biphasic
Endothelial

Incidence

Slits

Cells

Stroma

Commonest

++
+
-

Spindle
Spindle & epithelioid
Pleomorphic & round

++
Pseudoacinar
-

Least

Diagnosis:
Clinically:

Slowly growing over years before seeking an advice & misdiagnosed as benign (small size)
Usually male 32y a painful swelling below the mid-thigh
as it turns severely painful
1- Hyperintense fluid level
Soft to firm, nodular, tender

PXR:

2- Intemediat muscle signal

3- Hypointense fibrous signal

Soft tissue mass spot calcifications.... 30%

z joint space
MRI .......................................................... 30% Triple signal pattern on T2

Treatment:

Aggressive LIMB SALVAGE PROTOCOL wide resection + chemo + Radio vs

AMPUTATION

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Fibrous Benign
1. Calcifying aponeurotic fibroma
Slow Growing, painless mass ,ages 3-30
Xrays show faint mass with stippling
Histology shows fibrous tumour with some calcification and cartilage formation
50 % recurrence after excision
Resolves with maturity
2. Nodular fasciitis
Painful reactive rapidly enlarging lesion in a young person
50% in upper limbs
Histology- short irregular bundles and fascicles, only small amounts of mature collagen
Treat with excision with marginal resection
3. Palmar (Dupuytrens) and plantar (Ledderhosen) fibromatoses
4. Extraabdominal Desmoid tumour
Most locally invasive of the benign soft tissue tumours
Most common in adolescents and young adults
Rock hard on palpation
May be multiple lesions
Histologically well differentiated fibroblasts and abundant collagen
Infiltrates surrounding tissues
Surgery aims at excision with a wide margin
Local recurrence common

Fibrous Malignant
1. Fibrosarcoma
Enlarging painless mass
Age group 30-80
Usually 10cm in size before symptoms
Plain Xray usually normal unless encroaching on bone
MRI deepseated inhomogenous mass
Histology- fasciculated growth pattern with fusiform or spindle shaped cells, scanty
cytoplasm, indistinct borders separated by interwoven collagen, or herringbbone appearance
Treatment- Wide local excision. If > 5cm add radiation therapy, preop/postop/periop
2. Fibrohistiocytic
Dermatofibrosarcoma protruberans
Rare, nodular cutaneous tumour
Occurs in early adult life
Intermediate in grade
Recurs locally but only rarely metastasises
Treat with wide resection
3. Malignant fibrohistiocytoma
Similar to malignant fibrosarcoma
Histology slightly different with cartwheel pattern to the spindle and histiocytic cells
Treat as for fibrosarcoma

120

Benign Vascular Tumours

1. Haemangioma
Seen in children and adults
Can be cutaneous, subcutaneous, intramuscular
If large, patients complain due to symptoms of venous engorgement (aching)
Plain Xray can show small pleboliths
MR scan shows a heterogenous lesion with many small blood vessels
Treat nonoperatively if possible
Wide surgical resection if symptomatic, local recurrence rate high

Malignant Vascular Tumours

1. Haemangiopericytoma
Rare tumour of the pericytes of blood vessels
Can be benign or malignant and from intermediate to high grade
Slowly enlarging painless mass
Treatment based on grade of lesion
2. Angiosarcoma
Rare tumour resembles the endothelium of blood vessels
Treatment depends on grade and location of lesion

Benign Fatty Tumours

1. Lipomas
Subcutaneous/intramuscular or intermuscular tumours of mature fat
Most are not painful
Plain xrays may show a radioluscent region in the soft tissues
MR or CT scan show a well demarcated lesion with exactly the same signal as fat
If no symptoms and definite radiological diagnosis, leave alone
If mass growing or causing symptoms excise with a marginal line of resection
Recurrence uncommon
Subgroups are spindle cell cell lipoma and pleomorphic lipoma

Malignant Fatty Tumours

1. Liposarcomas
Malignant tumours with differentiation towards fatty tissue
Heterogenous group of tumours with the presence of signet ring type cells(lipoblasts) in
common
Lipoma like, Sclerosing, Inflammatory, Dedifferentiated, myxoid, round cell, pleomorphic
Range from low grade to high grade
LG liposarcomas - can be difficult to differentiate from benign lipoma
Low grade liposarcomas treated with wide local excision +/- radiotherapy
High grade liposarcomas treated with wide local excision + radiotherapy

121

Other Rare Sarcomas

1. Epithelioid Sarcoma
Nodular tumour occuring in the hands of young adults, buttock thigh, knee or foot
May ulcerate and mimic a granuloma or rheumatoid nodule
Lymph node metastases may occur
Histologically ovoid to polygonal with eosinophilic cytoplasm
Wide surgical excision required
2. Clear cell sarcoma
Slow growing painless mass in young adults
In region of tendons or aponeuroses
Treat with wide surgical resecion with adjuvant radiotherapy
3. Alveolar Cell Sarcoma
Most common in the anterior thigh
Treat with wide surgical excision and radiotherapy

122

Bone Metastases
Bone is the third most common site of metastatic disease
Cancers most likely to metastasise to bone:
1. Breast
2. Lung
3. Prostate
4. Thyroid
5. Kidney
6. Bowel
Carcinomas are much more likely to metastasise to bone than sarcomas
The axial skeleton is seeded more than the appendicular skeleton, partly due to the persistence
of red bone marrow in the former
The ribs, pelvis and spine are usually first affected
Batson's vertebral venous plexus allows cells to enter the vertebral circulation without first
passing through the lungs. The sluggish blood flow in this plexus is more conducive to tumour
survival, accounting for the high rate of prostate cancer metastasis to the spine.
CLINICALLY:

Pain, pathological fractures and hypercalcemia are the major sources of morbidity with bone
metastasis
Pain is the most common symptom found in 70% of patients with bone metastases. Pain is
caused by stretching of the periosteum by the tumour as well as nerve stimulation in the
endosteum
Pathological fractures are most common in breast cancer due to the lytic nature of the lesions.
They are uncommon in lung cancer due to short life span and rare in prostate cancer which
tend to be osteoblastic lesions
PXR

Lytic bone metastases must be greater than 1 cm and have destroyed 30-50% of the bone
density in order to be seen by x-ray
Metastatic bone lesions can be described as osteolytic, osteoblastic and mixed
The osteolytic lesions are most common where the destructive processes outstrip the laying
down of new bone
Osteoblastic lesions result from new bone growth that is stimulated by the tumour
WORKUP

Known Primary:
Bone Scan - Galasko reported that a period of two to eighteen months was necessary before a
lesion identified on bone scanning could be visualised on plain x-rays
FBC, Bone Biochemistry, LFT, Coagulation screen, G&S
Biopsy not necessary
Unknown Primary:
FBC, Bone Biochemistry, LFT, Coagulation screen, G&S
SPEP, TFT, PSA, Tumour markers, ESR, CRP
Bone Scan
CXR
CT chest & abdomen (or USS)
Biopsy lesion(s) for histology & microbiology (incl. TB)

123
TREATMENT

NORMALLY PALLIATIVE
RADIOTHERAPY:
o
o
o
o
o

useful for reducing bone pain and progression of tumour growth

90% will receive some relief, and 50% will receive near complete relief with between
20 to 40.5 gray of radiation
Complications: radiation induced osteonecrosis & y rate of stress # or nonunion
Lesions that do not represent a risk for fracture may be treated with radiation or by
appropriate chemotherapy directed at the tumour
Patients who have recurrence of pain because of biomechanical weakness should be
managed with operative stabilisation

BIPHOSPHONATES
o

o
o
o
o
o

Because of their down-regulation of bone resorption, bisphosphonates have become

attractive adjuvants for the reduction of the risk of pathological fracture stemming
from osteolytic lesions and for the treatment of bone pain
Studies of patients being managed for hypercalcemia have demonstrated a decrease in
bone pain with attainment of normal levels of calcium
Clodronate can be administered intravenously or orally, and although it affects bone
resorption it does not alter bone mineralization
Clodronate was found to reduce bone pain after a few months of therapy
The need for palliative DXT; and, potentially, the risk of pathological # z
Although bisphosphonates act quickly to reduce hypercalcemia, long-term
administration is necessary to treat widespread osteolytic disease.

PATHOL
OLOGICAL FRACTURE:

Pathological fractures have been reported to occur in 9-29% of patients who have bone
metastases, depending on the location of the lesion
High risk of pathological fracture =
1. > 50% loss of the cortex / shaft diameter on any view
2. Avulsion of the lesser trochanter is an indication of imminent hip fracture
3. (>2.5cm lesion)
4. MIRELS SCORING SYSTEM (CORR 1989) - a numerical score according to 4 variables:
location of the lesion
PXR appearance
the degree of pain
the size of the lesion
Mirels recommended: < 7 irradiation; > 7 operative treatment
Goals of surgery:
1. relief of pain
2. restoration of the ability to walk
3. preserve stability and function
4. an increased duration of survival
5. improved fracture-healing
Options:
o IM Nails + DXT entire bone
o Prosthesis - Epiphyseal fractures
o Endoprosthesis 4 extensive lesions
o major bone defects use PMMA
Emergency surgery is done for spinal metastasis in the hope of preserving neurological
function.
Postop radiotherapy should be considered in all cases, once wound healing has occurred.